Key Highlights:

• Castleman Disease can be classified into unicentric or multicentric, depending on the affected lymph nodes
• Unicentric affects only a single lymph node region, while multicentric affects multiple regions
• The symptoms of each variant differ, ranging from mild to severe and potentially life-threatening
• The disease is associated with the over-production of interleukin-6 (a protein produced by cells in response to injuries and infections), but the exact cause remains unknown
• Accurate diagnosis and timely treatment are critical for effective management of the disease

Introduction

Dealing with a rare medical condition can be tough. From the right diagnosis to effective care, it can be tricky. Castleman Disease (CD) is one such unusual illness. It has left many puzzled because of its hard-to-grasp nature and complex signs. But, what if we went deeper and really understood this disease? It would equip patients and their loved ones to better navigate the world of healthcare.

In this blog, we break down Castleman Disease – its types, signs, causes, and treatments. By the end of it, you’ll have a strong understanding of this rare disease that excites growth in the body’s cells. This knowledge can lead to better treatment and outcomes for those affected.

Overview of Castleman Disease

Definition and Types

Castleman Disease is a rare health issue tied to the immune system. An overactive immune system leads to bigger lymph nodes which can affect lymph nodes of any body region, imitating both benign and malignant conditions, including the neck, chest, abdomen, and pelvis. The disease can be classified into two types. Unicentric Castleman Disease (UCD) affects only a single lymph node region and is the more common presentation, Multicentric Castleman Disease (MCD) brings about inflamed lymph nodes in many body parts. 

Differences between Unicentric and Multicentric Castleman Disease

Let’s take a look at how the two main types of Castleman’s Disease differ:

Symptoms and Signs

Unicentric Castleman Disease Symptoms

People with Unicentric Castleman Disease may not show any signs at first (usually asymptomatic) and is usually discovered at radiological examinations performed for other reasons When symptoms do pop up, they are usually mild. They are linked to the growth of the lymph node. Common symptoms include:

• A lump under the skin in areas like the neck.
• Chest pressure or fullness, making it hard to breathe or swallow.
• Pain in the affected area

Multicentric Castleman Disease Symptoms

Multicentric Castleman Disease (MCD) is a severe condition that can be life-threatening, and represents the “dark side” of Castleman disease. HIV infection is an important risk and bad prognosis factor associated with MCD Boosted by many whole-body symptoms, it shows up as larger lymph nodes. Such symptoms include:

• Fever
• Tiredness
• Night sweats
• Weight loss without trying
• Swelling of the liver and spleen

MCD can also be divided into two more subtypes: Idiopathic Multicentric Castleman Disease (iMCD) and Kaposi Sarcoma-Associated Herpesvirus Multicentric Castleman Disease (KSHV-MCD).

Researchers have found that your risk of acquiring cancer, especially lymphoma (a cancer of the lymphatic system), is likely to increase by Castleman disease. Consult your doctor and he will keep a close eye on your condition and adapt your treatments wherever feasible to avoid complications.

Dr. Siddharth Gupta, B.A.M.S, M.D

When to See a Doctor

Identifying Red Flags and Atypical Symptoms

Seeing a doctor is important when you come across unusual symptoms. An example would be a large lymph node area, e.g., neck or underarm. Other signs needing quick action include feelings of fullness in the chest, fever, fatigue, and weight loss without trying. Spotting these warning signs can lead to a quick diagnosis and helpful intervention.

Importance of Timely Consultation

Recognize how important it is to get medical help on time:

In my experience, the symptoms of Castleman disease are comparable to those of other infections, such as the flu. To rule out certain illnesses, your doctor might do a number of tests. To ascertain the form of Castleman’s illness, they will do additional testing such as biopsy.

Dr. Rajeev Singh, BAMS

Causes and Risk Factors

1. Aetiology

Researchers are still figuring out the exact cause. But, they believe that it comes from a faulty immune system response. This drives an overgrowth of certain cells in lymphoid organs. The growth could be brought about by ongoing mild inflammation, viral infections, abnormal cytokine control, or new blood vessel growth. Certain autoimmune conditions, cancers or infections can present with changes in lymph nodes or clinical features that closely resemble Castleman disease. It’s unknown whether Castleman disease should be considered an autoimmune disease, cancer or infectious disease.

2. Associated Viruses and Conditions

Human immunodeficiency virus (HIV) and human herpesvirus 8 (HHV-8), a common virus [Most people who have it don’t get sick. You’re more likely to have HHV-8-associated MCD if you’re HIV positive or if you have another condition that makes you more susceptible to infections (immunocompromised)]are linked to Multicentric Castleman Disease, mainly its KSHV-MCD variant.

3. Genetic and Environmental Factors

Even though clear genetic links haven’t been found yet, some cases hint at a possible family link. Environmental factors might also play a role, but we don’t know yet how much impact they have.

Take a look at this table showcasing possible causal factors:

Who does Castleman disease affect?

Castleman disease can affect anyone. UCD is more commonly diagnosed in people in their 30s and 40s. MCD is more common in people in their 50s and 60s. While UCD affects people equally regardless of sex, MCD is slightly more common in men and people assigned male at birth (AMAB).

How common is Castleman disease?

Castleman disease is rare, with approximately 4,300 to 5,200 new cases diagnosed in the United States each year. About 75% of these cases are UCD, and the remaining 25% are MCD.

Complications and Related Conditions

1. KSHV Inflammatory Cytokine Syndrome

This syndrome is shown by high levels of inflammation, fever, weight loss and severe organ failure. It is linked with the KSHV-MCD type of Castleman’s disease, usually as a result of sudden release of inflammatory compounds, causing tissue inflammation and damage.

2. POEMS Syndrome

POEMS Syndrome is a rare blood disorder that damages nerves and affects other body systems. POEMS stands for Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes. This blood disorder is linked to a subtype of Multicentric Castleman Disease.

3. Lymphoproliferative Disorders

Some patients with Castleman Disease could get other diseases, like non-Hodgkin lymphoma.

The next table highlights, at a glance, complications and related conditions:

Diagnosis and Screening

1. Clinical Presentation

Medical history, physical exam, and imaging techniques, such as CT scans or MRIs, help identify large lymph nodes. This can guide further tests.

2. Pathological Features and Criteria

Checking affected lymph nodes is vital for diagnosing Castleman’s Disease, as different types have different features.

Unicentric Castleman Disease

This type usually shows up as large lymph nodes with a high number of certain cell types and a wide mantle zone. Small germinal centres may also be present.

Multicentric Castleman Disease

MCD can show up with many tissue features, including high blood vessel growth and hyalinization in certain zones. Plasma cell infiltration and faulty follicular dendritic cells might occur too.

3. Diagnostic Tests and Imaging

In addition to looking at tissue, blood tests and imaging studies like CT scans, MRIs and PET scan (Position Emission Tomography) can help diagnose Castleman Disease. This also guides to its extent.

4. Differential Diagnosis and Overlapping Disorders

Diseases that can act like Castleman Disease include lymphomas, solid tumors, autoimmune disorders, and other diseases leading to swollen lymph nodes. A detailed diagnostic test is needed to rule out these possible diagnoses.

Go through this table to learn about the main diagnostic methods for Castleman Disease:

Treatment and Management

1. Unicentric Castleman Disease Treatment

The standard treatment for Unicentric Castleman Disease is full surgical removal of the affected lymph node or group. This can often lead to a cure. Depending on where the lymph nodes are located or if they’re too big, you may need to receive radiation therapy or immunotherapy before surgery for UCD. These therapies shrink the growths on your lymph nodes, making them easier to remove. If surgeons can’t remove the tumour and you don’t have symptoms, your provider may prefer to monitor your tumour instead of treating it immediately. If surgery isn’t an option and you have symptoms, your provider may recommend treatments for multicentric Castleman disease.

2. Multicentric Castleman Disease Treatment

MCD is more difficult to treat than UCD. Because the multicentric type is widespread, healthcare providers don’t typically use surgery or radiation to treat it. Instead, treatment depends on whether you have HHV-8 and how severe the disease is. Treatment for MCD typically involves many therapies aiming at the root cause or disease path, such as antiviral drugs (for KSHV-MCD), monoclonal-antibody treatments (targeting interleukin-6 or CD20), chemotherapy, and palliative care. Some patients might need ongoing treatment to manage symptoms and avoid complications.

iMCD and iMCD-TAFRO

Patients with idiopathic multicentric Castleman Disease and iMCD-TAFRO might be treated with interleukin-6 inhibitors like tocilizumab and siltuximab. Other immune treatment methods and palliative care measures may also be essential.

KSHV-MCD and KSHV-Associated Lymphoproliferations

Antiviral drugs like ganciclovir, foscarnet, and cidofovir can help manage KSHV-MCD and KSHV clone disorders. More steps, like radiation therapy and targeted therapies, may also be needed for some cases.

What complications are associated with Castleman disease?

Castleman disease increases your chances of developing cancer, including lymphoma (cancer of the lymph system).

MCD can increase your risk of developing infections that can damage organs and even be life-threatening without treatment.

1. What are the risk factors for Castleman disease?

There aren’t any known risk factors for UCD or iMCD. People with the human immunodeficiency virus (HIV) or a weakened immune system are at higher risk of developing HHV-8-associated MCD.

2. Can Castleman disease be prevented?

There aren’t any known risk factors for most types of Castleman disease, so there’s nothing you can do to prevent them.

3. Monitoring and Follow-Up

Patients with Castleman Disease need regular check-ups with their healthcare provider. This monitors disease progress, how the treatment is doing, and possible complications.

Have a look at this table of treatment options for Castleman Disease:

Quality of Life and Living with Castleman Disease

Coping Mechanisms and Support Resources

Support groups like the Castleman Disease Collaborative Network (CDCN) offer valuable resources. These can help patients and their families navigate this rare disease. Professional talking therapies may also be helpful in managing stress, worry, and sadness tied to living with Castleman Disease.

Long-Term Prognosis and Outlook

The future outlook for people with Castleman Disease changes based on the type and severity of the disorder. Unicentric Castleman Disease often has a good outcome after surgical removal. On the other hand, managing Multicentric Castleman Disease can be more complex. It often requires ongoing treatment and monitoring.

Future Research and Development

1. Advances in Pathogenesis Understanding

We need ongoing research into the root causes and workings of Castleman Disease. This will help create better tests and treatments. Getting a firmer grasp on our immune system, cytokine control, and genetics may pave the way for new treatments for this rare disorder.

2. Diagnostic Innovations

Better existing diagnostic tools and finding new disease markers for Castleman Disease will aid early detection. It will also help tell it apart from other lymph proliferative diseases. This leads to improved outcomes for patients.

3. Therapeutic Improvements

Research is driving the development of new treatments targeting key pathways involved in Castleman Disease. It can lead to more effective treatment choices and fewer side effects. This can greatly enhance the patients’ lives.

Also Read: What is Reiki Healing: A Comprehensive Understanding Through Research

Conclusion

Castleman Disease is a complex and rare disorder that excites cell growth. It has a diverse range of signs, from the harmless Unicentric Castleman Disease to the life-threatening Multicentric Castleman Disease. A correct diagnosis, quick steps, and proper care are crucial for fighting this hard to catch disease and reducing its impact on the patient’s life, and that of their loved ones. With ongoing research, we can hope for a future where we better understand the workings, diagnosis, and treatment of Castleman Disease. This can provide better options and improved outcomes for patients and their families.

FAQ (Frequently Asked Questions)

References:

1. National Center for Biotechnology Information (NCBI). Castleman Disease [Internet]. [cited 2023 Oct 3]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK576394
2. National Center for Biotechnology Information (NCBI). Case records of the Massachusetts General Hospital: Case No. 40231 [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=13165944
3. National Center for Biotechnology Information (NCBI). Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=4551306
4. National Center for Biotechnology Information (NCBI). Multicentric giant lymph node hyperplasia. A report of seven cases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=2412685
5. National Center for Biotechnology Information (NCBI). International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33284946
6. National Center for Biotechnology Information (NCBI). International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7724917
7. National Center for Biotechnology Information (NCBI). Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=7632932
8. National Center for Biotechnology Information (NCBI). KSHV-associated multicentric Castleman disease: A tangle of different entities requiring multitarget treatment strategies [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=24771491
9. National Center for Biotechnology Information (NCBI). Characteristics and outcomes of KSHV-associated multicentric Castleman disease with or without other KSHV diseases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33720337
10. National Center for Biotechnology Information (NCBI). Characteristics and outcomes of KSHV-associated multicentric Castleman disease with or without other KSHV diseases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7993110
11. National Center for Biotechnology Information (NCBI). HIV-associated lymphomas and gamma-herpesviruses [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=18955561
12. National Center for Biotechnology Information (NCBI). Clinical Manifestations of Kaposi Sarcoma Herpesvirus Lytic Activation: Multicentric Castleman Disease (KSHV-MCD) and the KSHV Inflammatory Cytokine Syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22403576
13. National Center for Biotechnology Information (NCBI). Clinical Manifestations of Kaposi Sarcoma Herpesvirus Lytic Activation: Multicentric Castleman Disease (KSHV–MCD) and the KSHV Inflammatory Cytokine Syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3291870
14. National Center for Biotechnology Information (NCBI). Clinical implications of idiopathic multicentric castleman disease among Japanese: a report of 28 cases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=18499694
15. National Center for Biotechnology Information (NCBI). Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10339470
16. National Center for Biotechnology Information (NCBI). KSHV/HHV8-positive large B-cell lymphomas and associated diseases: a heterogeneous group of lymphoproliferative processes with significant clinicopathological overlap [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31527708
17. National Center for Biotechnology Information (NCBI). How I treat HIV-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=20688959
18. National Center for Biotechnology Information (NCBI). Overview of Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32106302
19. National Center for Biotechnology Information (NCBI). Multicentric Castleman’s disease in Malawi [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=25241721
20. National Center for Biotechnology Information (NCBI). Multicentric Castleman’s disease in Malawi [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4470481
21. National Center for Biotechnology Information (NCBI). Epidemiology of Castleman Disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29157611
22. National Center for Biotechnology Information (NCBI). The full spectrum of Castleman disease: 273 patients studied over 20 years [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29143319
23. National Center for Biotechnology Information (NCBI). International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30181172
24. National Center for Biotechnology Information (NCBI). International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6238190
25. National Center for Biotechnology Information (NCBI). Clinicopathologic characteristics of 342 patients with multicentric Castleman disease in Japan [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31829070
26. National Center for Biotechnology Information (NCBI). Characteristics and survival for HIV-associated multicentric Castleman disease in Malawi [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=26242311
27. National Center for Biotechnology Information (NCBI). Characteristics and survival for HIV-associated multicentric Castleman disease in Malawi [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4524888
28. National Center for Biotechnology Information (NCBI). Paediatric Castleman disease: report of seven cases and review of the literature [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10445341
29. National Center for Biotechnology Information (NCBI). The French paediatric cohort of Castleman disease: a retrospective report of 23 patients [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32303241
30. National Center for Biotechnology Information (NCBI). The French paediatric cohort of Castleman disease: a retrospective report of 23 patients [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7164260
31. National Center for Biotechnology Information (NCBI). Multicentric Castleman disease in an HHV8-infected child born to consanguineous parents with systematic review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22157133
32. National Center for Biotechnology Information (NCBI). Multicentric Castleman Disease in an HHV8-Infected Child Born to Consanguineous Parents With Systematic Review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3273364
33. National Center for Biotechnology Information (NCBI). Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31708515
34. National Center for Biotechnology Information (NCBI). Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6954167
35. National Center for Biotechnology Information (NCBI). Use of a claims database to characterize and estimate the incidence rate for Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=25120049
36. National Center for Biotechnology Information (NCBI). Update on Kaposi sarcoma-associated herpesvirus (KSHV or HHV8) – review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32681788
37. National Center for Biotechnology Information (NCBI). Differences in incidence and trends of haematological malignancies in Japan and the United States [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=24245986
38. National Center for Biotechnology Information (NCBI). The role of immune suppression and HHV-8 in the increasing incidence of HIV-associated multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=19179554
39. National Center for Biotechnology Information (NCBI). Clinical spectrum and survival analysis of 145 cases of HIV-negative Castleman’s disease: renal function is an important prognostic factor [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27029894
40. National Center for Biotechnology Information (NCBI). Clinical spectrum and survival analysis of 145 cases of HIV-negative Castleman’s disease: renal function is an important prognostic factor [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4814900
41. National Center for Biotechnology Information (NCBI). HIV-associated Kaposi sarcoma and related diseases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28609402
42. National Center for Biotechnology Information (NCBI). Kaposi sarcoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30705286
43. National Center for Biotechnology Information (NCBI). Kaposi’s sarcoma and its associated herpesvirus [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=20865011
44. National Center for Biotechnology Information (NCBI). Kaposi’s sarcoma herpesvirus/ Human herpesvirus-8 (KSHV/HHV8), and the oncogenesis of Kaposi’s sarcoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4721662
45. National Center for Biotechnology Information (NCBI). The clinical spectrum of Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22791417
46. National Center for Biotechnology Information (NCBI). The Clinical Spectrum of Castleman’s Disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3900496
47. National Center for Biotechnology Information (NCBI). Multicentric angiofollicular lymph node hyperplasia: a clinicopathologic study of 16 cases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=2579015
48. National Center for Biotechnology Information (NCBI). Monoclonality and cytogenetic abnormalities in hyaline vascular Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=24201121
49. National Center for Biotechnology Information (NCBI). Recurrent PDGFRB mutations in unicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30607019
50. National Center for Biotechnology Information (NCBI). Recurrent PDGFRB mutations in unicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6484698
51. National Center for Biotechnology Information (NCBI). Virome capture sequencing does not identify active viral infection in unicentric and idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31242229
52. National Center for Biotechnology Information (NCBI). Virome capture sequencing does not identify active viral infection in unicentric and idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6594611
53. National Center for Biotechnology Information (NCBI). HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=24622327
54. National Center for Biotechnology Information (NCBI). Novel somatic alterations in unicentric and idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=34431136
55. National Center for Biotechnology Information (NCBI). Next-generation sequencing of idiopathic multicentric and unicentric Castleman disease and follicular dendritic cell sarcomas [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29496669
56. National Center for Biotechnology Information (NCBI). Next-generation sequencing of idiopathic multicentric and unicentric Castleman disease and follicular dendritic cell sarcomas [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5851414
57. National Center for Biotechnology Information (NCBI). DNMT3A haploinsufficiency causes dichotomous DNA methylation defects at enhancers in mature human immune cells [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33970190
58. National Center for Biotechnology Information (NCBI). DNMT3A haploinsufficiency causes dichotomous DNA methylation defects at enhancers in mature human immune cells [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8111463
59. National Center for Biotechnology Information (NCBI). Genetic basis for iMCD-TAFRO [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32051554
60. National Center for Biotechnology Information (NCBI). Genetic basis for iMCD-TAFRO [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7148173
61. National Center for Biotechnology Information (NCBI). A novel <em>FAS</em> mutation with variable expressivity in a family with unicentric and idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30404775
62. National Center for Biotechnology Information (NCBI). A novel FAS mutation with variable expressivity in a family with unicentric and idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234382
63. National Center for Biotechnology Information (NCBI). Cytokine Storm [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33264547
64. National Center for Biotechnology Information (NCBI). Cytokine Storm [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7727315
65. National Center for Biotechnology Information (NCBI). Castleman disease in the 21st century: an update on diagnosis, assessment, and therapy [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=20864917
66. National Center for Biotechnology Information (NCBI). Effects of recombinant human interleukin-6 in cancer patients: a phase I-II study [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=8068939
67. National Center for Biotechnology Information (NCBI). Elevated levels of interleukin-1 beta (IL-1 beta) and IL-6 in serum and increased production of IL-1 beta mRNA in lymph nodes of patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=8167341
68. National Center for Biotechnology Information (NCBI). Multicentric Castleman’s disease with an increased serum level of macrophage colony-stimulating factor [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=9092689
69. National Center for Biotechnology Information (NCBI). Expression of vascular endothelial growth factor in sera and lymph nodes of the plasma cell type of Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10086783
70. National Center for Biotechnology Information (NCBI). ACCELERATE: A Patient-Powered Natural History Study Design Enabling Clinical and Therapeutic Discoveries in a Rare Disorder [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33377129
71. National Center for Biotechnology Information (NCBI). ACCELERATE: A Patient-Powered Natural History Study Design Enabling Clinical and Therapeutic Discoveries in a Rare Disorder [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7762771
72. National Center for Biotechnology Information (NCBI). Plasma proteomics identifies a ‘chemokine storm’ in idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29675946
73. National Center for Biotechnology Information (NCBI). Identifying and targeting pathogenic PI3K/AKT/mTOR signaling in IL-6-blockade-refractory idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31408438
74. National Center for Biotechnology Information (NCBI). Identifying and targeting pathogenic PI3K/AKT/mTOR signaling in IL-6 blockade–refractory idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6763254
75. National Center for Biotechnology Information (NCBI). The Kaposi sarcoma-associated herpesvirus (KSHV) induces cellular interleukin 6 expression: role of the KSHV latency-associated nuclear antigen and the AP1 response element [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=11781250
76. National Center for Biotechnology Information (NCBI). Type I IFN response associated with mTOR activation in the TAFRO subtype of idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253017
77. National Center for Biotechnology Information (NCBI). Discovery and validation of a novel subgroup and therapeutic target in idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=34438448
78. National Center for Biotechnology Information (NCBI). Discovery and validation of a novel subgroup and therapeutic target in idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8525223
79. National Center for Biotechnology Information (NCBI). Increased mTOR activation in idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32206779
80. National Center for Biotechnology Information (NCBI). Increased mTOR activation in idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7205815
81. National Center for Biotechnology Information (NCBI). Mediterranean fever gene variants modify clinical phenotypes of idiopathic multi-centric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=34096620
82. National Center for Biotechnology Information (NCBI). Mediterranean fever gene variants modify clinical phenotypes of idiopathic multi‐centric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8663796
83. National Center for Biotechnology Information (NCBI). Idiopathic multicentric Castleman disease with novel heterozygous Ile729Met mutation in exon 10 of familial Mediterranean fever gene [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32830263
84. National Center for Biotechnology Information (NCBI). Successful canakinumab treatment for activated innate response in idiopathic Castleman’s disease with multiple heterozygous MEFV exon 2 variants [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32735870
85. National Center for Biotechnology Information (NCBI). Kaposi sarcoma-associated herpesvirus infects monotypic (IgM lambda) but polyclonal naive B cells in Castleman disease and associated lymphoproliferative disorders [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=11264181
86. National Center for Biotechnology Information (NCBI). Distribution of human herpesvirus-8 latently infected cells in Kaposi’s sarcoma, multicentric Castleman’s disease, and primary effusion lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10200299
87. National Center for Biotechnology Information (NCBI). Distribution of human herpesvirus-8 latently infected cells in Kaposi’s sarcoma, multicentric Castleman’s disease, and primary effusion lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC16369
88. National Center for Biotechnology Information (NCBI). Differential viral protein expression in Kaposi’s sarcoma-associated herpesvirus-infected diseases: Kaposi’s sarcoma, primary effusion lymphoma, and multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10702388
89. National Center for Biotechnology Information (NCBI). Differential Viral Protein Expression in Kaposi’s Sarcoma-Associated Herpesvirus-Infected Diseases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1876837
90. National Center for Biotechnology Information (NCBI). KSHV induces immunoglobulin rearrangements in mature B lymphocytes [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29659614
91. National Center for Biotechnology Information (NCBI). KSHV induces immunoglobulin rearrangements in mature B lymphocytes [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5919685
92. National Center for Biotechnology Information (NCBI). The viral interferon regulatory factors of kaposi’s sarcoma-associated herpesvirus differ in their inhibition of interferon activation mediated by toll-like receptor 3 [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=23115281
93. National Center for Biotechnology Information (NCBI). The Viral Interferon Regulatory Factors of Kaposi’s Sarcoma-Associated Herpesvirus Differ in Their Inhibition of Interferon Activation Mediated by Toll-Like Receptor 3 [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3554052
94. National Center for Biotechnology Information (NCBI). Kaposi sarcoma-associated herpesvirus: immunobiology, oncogenesis, and therapy [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27584730
95. National Center for Biotechnology Information (NCBI). Kaposi sarcoma–associated herpesvirus: immunobiology, oncogenesis, and therapy [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5004954
96. National Center for Biotechnology Information (NCBI). High-dose zidovudine plus valganciclovir for Kaposi sarcoma herpesvirus-associated multicentric Castleman disease: a pilot study of virus-activated cytotoxic therapy [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=21487108
97. National Center for Biotechnology Information (NCBI). High-dose zidovudine plus valganciclovir for Kaposi sarcoma herpesvirus-associated multicentric Castleman disease: a pilot study of virus-activated cytotoxic therapy [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3143547
98. National Center for Biotechnology Information (NCBI). A viral kinase mimics S6 kinase to enhance cell proliferation [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27342859
99. National Center for Biotechnology Information (NCBI). A viral kinase mimics S6 kinase to enhance cell proliferation [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4948314
100. National Center for Biotechnology Information (NCBI). Human herpesvirus-encoded kinase induces B cell lymphomas in vivo [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29733294
101. National Center for Biotechnology Information (NCBI). Human herpesvirus–encoded kinase induces B cell lymphomas in vivo [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5983348
102. National Center for Biotechnology Information (NCBI). Heterogeneity of viral IL-6 expression in HHV-8-associated diseases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10438372
103. National Center for Biotechnology Information (NCBI). Expression of a virus-derived cytokine, KSHV vIL-6, in HIV-seronegative Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=9403701
104. National Center for Biotechnology Information (NCBI). Cellular tropism and viral interleukin-6 expression distinguish human herpesvirus 8 involvement in Kaposi’s sarcoma, primary effusion lymphoma, and multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10196314
105. National Center for Biotechnology Information (NCBI). Cellular Tropism and Viral Interleukin-6 Expression Distinguish Human Herpesvirus 8 Involvement in Kaposi’s Sarcoma, Primary Effusion Lymphoma, and Multicentric Castleman’s Disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC104197
106. National Center for Biotechnology Information (NCBI). Human and viral interleukin-6 and other cytokines in Kaposi sarcoma herpesvirus-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=24174627
107. National Center for Biotechnology Information (NCBI). Human and viral interleukin-6 and other cytokines in Kaposi sarcoma herpesvirus-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3868925
108. National Center for Biotechnology Information (NCBI). High levels of human herpesvirus 8 viral load, human interleukin-6, interleukin-10, and C reactive protein correlate with exacerbation of multicentric castleman disease in HIV-infected patients [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10979949
109. National Center for Biotechnology Information (NCBI). Serum viral interleukin-6 in AIDS-related multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=11307774
110. National Center for Biotechnology Information (NCBI). Kaposi’s sarcoma-associated herpesvirus encoded vFLIP induces cellular IL-6 expression: the role of the NF-kappaB and JNK/AP1 pathways [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=12776188
111. National Center for Biotechnology Information (NCBI). Rta of the human herpesvirus 8/Kaposi sarcoma-associated herpesvirus up-regulates human interleukin-6 gene expression [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=12176919
112. National Center for Biotechnology Information (NCBI). HHV8/KSHV-Positive Lymphoproliferative Disorders and the Spectrum of Plasmablastic and Plasma Cell Neoplasms: 2015 SH/EAHP Workshop Report-Part 3 [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28395104
113. National Center for Biotechnology Information (NCBI). HHV8/KSHV-Positive Lymphoproliferative Disorders and the Spectrum of Plasmablastic and Plasma Cell Neoplasms [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6248411
114. National Center for Biotechnology Information (NCBI). Contribution of viral and cellular cytokines to Kaposi’s sarcoma-associated herpesvirus pathogenesis [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=18319288
115. National Center for Biotechnology Information (NCBI). Contribution of viral and cellular cytokines to Kaposi’s sarcoma-associated herpesvirus pathogenesis [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2538598
116. National Center for Biotechnology Information (NCBI). HHV-8 infection status of AIDS-unrelated and AIDS-associated multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=11696169
117. National Center for Biotechnology Information (NCBI). Clinical features and treatment of multicentric castleman’s disease : a retrospective study of 21 Japanese patients at a single institute [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=23801137
118. National Center for Biotechnology Information (NCBI). Monocyte-derived human B-cell growth factor identified as interferon-beta 2 (BSF-2, IL-6) [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=2829354
119. National Center for Biotechnology Information (NCBI). Critical cytoplasmic region of the interleukin 6 signal transducer gp130 is conserved in the cytokine receptor family [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=1662392
120. National Center for Biotechnology Information (NCBI). Critical cytoplasmic region of the interleukin 6 signal transducer gp130 is conserved in the cytokine receptor family. [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC53132
121. National Center for Biotechnology Information (NCBI). Kaposi’s sarcoma-associated human herpesvirus-8 encodes homologues of macrophage inflammatory protein-1 and interleukin-6 [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=9055855
122. National Center for Biotechnology Information (NCBI). Viral IL-6-induced cell proliferation and immune evasion of interferon activity [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=12434062
123. National Center for Biotechnology Information (NCBI). KSHV: pathways to tumorigenesis and persistent infection [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=24373311
124. National Center for Biotechnology Information (NCBI). KSHV: Pathways to Tumorigenesis and Persistent Infection [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4104069
125. National Center for Biotechnology Information (NCBI). Kaposi’s Sarcoma-Associated Herpesvirus Interleukin-6 Modulates Endothelial Cell Movement by Upregulating Cellular Genes Involved in Migration [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=26646010
126. National Center for Biotechnology Information (NCBI). Kaposi’s Sarcoma-Associated Herpesvirus Interleukin-6 Modulates Endothelial Cell Movement by Upregulating Cellular Genes Involved in Migration [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4676281
127. National Center for Biotechnology Information (NCBI). Increased expression of vascular endothelial growth factor (VEGF) in Castleman’s disease: proposed pathomechanism of vascular proliferation in the affected lymph node [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10830746
128. National Center for Biotechnology Information (NCBI). Angiogenesis and hematopoiesis induced by Kaposi’s sarcoma-associated herpesvirus-encoded interleukin-6 [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=10361100
129. National Center for Biotechnology Information (NCBI). Colocalization of the viral interleukin-6 with latent nuclear antigen-1 of human herpesvirus-8 in endothelial spindle cells of Kaposi’s sarcoma and lymphoid cells of multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=11172301
130. National Center for Biotechnology Information (NCBI). Extracellular vesicles from Kaposi Sarcoma-associated herpesvirus lymphoma induce long-term endothelial cell reprogramming [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30716130
131. National Center for Biotechnology Information (NCBI). Extracellular vesicles from Kaposi Sarcoma-associated herpesvirus lymphoma induce long-term endothelial cell reprogramming [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6361468
132. National Center for Biotechnology Information (NCBI). Pre-micro RNA signatures delineate stages of endothelial cell transformation in Kaposi sarcoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=19381257
133. National Center for Biotechnology Information (NCBI). Pre-Micro RNA Signatures Delineate Stages of Endothelial Cell Transformation in Kaposi Sarcoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2663814
134. National Center for Biotechnology Information (NCBI). Molecular mimicry of human cytokine and cytokine response pathway genes by KSHV [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=8939871
135. National Center for Biotechnology Information (NCBI). Reactivation of the p53 pathway as a treatment modality for KSHV-induced lymphomas [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=17364023
136. National Center for Biotechnology Information (NCBI). Reactivation of the p53 pathway as a treatment modality for KSHV-induced lymphomas [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1810577
137. National Center for Biotechnology Information (NCBI). An interleukin-6-related systemic inflammatory syndrome in patients co-infected with Kaposi sarcoma-associated herpesvirus and HIV but without Multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=20583924
138. National Center for Biotechnology Information (NCBI). Runaway Kaposi Sarcoma-associated herpesvirus replication correlates with systemic IL-10 levels [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31629226
139. National Center for Biotechnology Information (NCBI). Vironome of Kaposi sarcoma associated herpesvirus-inflammatory cytokine syndrome in an AIDS patient reveals co-infection of human herpesvirus 8 and human herpesvirus 6A [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22925337
140. National Center for Biotechnology Information (NCBI). Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31016730
141. National Center for Biotechnology Information (NCBI). UCD with MCD-like inflammatory state: surgical excision is highly effective [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33570636
142. National Center for Biotechnology Information (NCBI). UCD with MCD-like inflammatory state: surgical excision is highly effective [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7805307
143. National Center for Biotechnology Information (NCBI). Clinical Analysis of Unicentric Castleman’s Disease with Paraneoplastic Pemphigus and Bronchiolitis Obliterans [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28877826
144. National Center for Biotechnology Information (NCBI). International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28087540
145. National Center for Biotechnology Information (NCBI). International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5364342
146. National Center for Biotechnology Information (NCBI). Expression of human herpesvirus-8 oncogene and cytokine homologues in an HIV-seronegative patient with multicentric Castleman’s disease and primary effusion lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=9881964
147. National Center for Biotechnology Information (NCBI). The clinical application of plasma Kaposi sarcoma herpesvirus viral load as a tumour biomarker: results from 704 patients [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=26111246
148. National Center for Biotechnology Information (NCBI). Clinical Features and Outcomes of Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV)-associated Inflammation: Prospective Characterization of KSHV Inflammatory Cytokine Syndrome (KICS) [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=26658701
149. National Center for Biotechnology Information (NCBI). Viral, immunologic, and clinical features of primary effusion lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30782610
150. National Center for Biotechnology Information (NCBI). Viral, immunologic, and clinical features of primary effusion lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6473499
151. National Center for Biotechnology Information (NCBI). Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28880697
152. National Center for Biotechnology Information (NCBI). Elevated serum interferon γ-induced protein 10 kDa is associated with TAFRO syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28205564
153. National Center for Biotechnology Information (NCBI). Elevated serum interferon γ-induced protein 10 kDa is associated with TAFRO syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5304226
154. National Center for Biotechnology Information (NCBI). 18F-fluorodeoxyglucose Positron Emission Tomography in Kaposi Sarcoma Herpesvirus-Associated Multicentric Castleman Disease: Correlation With Activity, Severity, Inflammatory and Virologic Parameters [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=25828248
155. National Center for Biotechnology Information (NCBI). 18 F-fluorodeoxyglucose Positron Emission Tomography in Kaposi Sarcoma Herpesvirus–Associated Multicentric Castleman Disease: Correlation With Activity, Severity, Inflammatory and Virologic Parameters [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4577043
156. National Center for Biotechnology Information (NCBI). Lymphomas occurring specifically in HIV-infected patients: from pathogenesis to pathology [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=23999127
157. National Center for Biotechnology Information (NCBI). Immunophenotypic analysis of the Kaposi sarcoma herpesvirus (KSHV; HHV-8)-infected B cells in HIV+ multicentric Castleman disease (MCD) [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=18983461
158. National Center for Biotechnology Information (NCBI). Multicentric Castleman’s disease in HIV infection: a clinical and pathological study of 20 patients [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=8924253
159. National Center for Biotechnology Information (NCBI). Castleman disease: an update on classification and the spectrum of associated lesions [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=19546611
160. National Center for Biotechnology Information (NCBI). CD20 expression in the HHV-8-infected lymphoid cells in multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=19723153
161. National Center for Biotechnology Information (NCBI). Lymph nodes involved by multicentric Castleman disease among HIV-positive individuals are often involved by Kaposi sarcoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=18469709
162. National Center for Biotechnology Information (NCBI). Idiopathic multicentric Castleman’s disease: a systematic literature review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27063975
163. National Center for Biotechnology Information (NCBI). Interleukin-6 receptor polymorphism is prevalent in HIV-negative Castleman Disease and is associated with increased soluble interleukin-6 receptor levels [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=23372742
164. National Center for Biotechnology Information (NCBI). Interleukin-6 Receptor Polymorphism Is Prevalent in HIV-negative Castleman Disease and Is Associated with Increased Soluble Interleukin-6 Receptor Levels [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3553080
165. National Center for Biotechnology Information (NCBI). Epidemiology of Castleman disease associated with AA amyloidosis: description of 2 new cases and literature review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31364863
166. National Center for Biotechnology Information (NCBI). Clinical and immunological findings in 104 cases of paraneoplastic pemphigus [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=26358412
167. National Center for Biotechnology Information (NCBI). Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30128272
168. National Center for Biotechnology Information (NCBI). Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6098213
169. National Center for Biotechnology Information (NCBI). Development of vascular neoplasia in Castleman’s disease. Report of seven cases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=2356921
170. National Center for Biotechnology Information (NCBI). Localized mediastinal lymphnode hyperplasia resembling thymoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=13356266
171. National Center for Biotechnology Information (NCBI). Unicentric Castleman’s disease complicated by systemic AA amyloidosis: a curable disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=11937647
172. National Center for Biotechnology Information (NCBI). Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=2788466
173. National Center for Biotechnology Information (NCBI). Siltuximab for multicentric Castleman’s disease: a randomised, double-blind, placebo-controlled trial [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=25042199
174. National Center for Biotechnology Information (NCBI). Analysis of Inflammatory and Anemia-Related Biomarkers in a Randomized, Double-Blind, Placebo-Controlled Study of Siltuximab (Anti-IL6 Monoclonal Antibody) in Patients With Multicentric Castleman Disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=26124203
175. National Center for Biotechnology Information (NCBI). Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=15998837
176. National Center for Biotechnology Information (NCBI). Predictors of response to anti-IL6 monoclonal antibody therapy (siltuximab) in idiopathic multicentric Castleman disease: secondary analyses of phase II clinical trial data [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30203839
177. National Center for Biotechnology Information (NCBI). Insufficient evidence exists to use histopathologic subtype to guide treatment of idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32894785
178. National Center for Biotechnology Information (NCBI). A case of multicentric Castleman’s disease associated with advanced systemic amyloidosis treated with chemotherapy and anti-CD20 monoclonal antibody [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=12898190
179. National Center for Biotechnology Information (NCBI). Efficacy of rituximab in an aggressive form of multicentric Castleman disease associated with immune phenomena [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=15795923
180. National Center for Biotechnology Information (NCBI). Long-term remission in HIV-negative patients with multicentric Castleman’s disease using rituximab [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=16405432
181. National Center for Biotechnology Information (NCBI). Mixed variant multicentric Castleman disease treated with rituximab: case report [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=20921905
182. National Center for Biotechnology Information (NCBI). Adrenalitis and anasarca in idiopathic multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33965090
183. National Center for Biotechnology Information (NCBI). Tocilizumab for the treatment of TAFRO syndrome: a systematic literature review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32964342
184. National Center for Biotechnology Information (NCBI). Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32970275
185. National Center for Biotechnology Information (NCBI). 2019 Updated diagnostic criteria and disease severity classification for TAFRO syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=31782045
186. National Center for Biotechnology Information (NCBI). How I treat POEMS syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22547581
187. National Center for Biotechnology Information (NCBI). How I treat POEMS syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3425020
188. National Center for Biotechnology Information (NCBI). Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M‐protein, and skin changes) syndrome [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7389818
189. National Center for Biotechnology Information (NCBI). European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30038381
190. National Center for Biotechnology Information (NCBI). Prospective study of rituximab in chemotherapy-dependent human immunodeficiency virus associated multicentric Castleman’s disease: ANRS 117 CastlemaB Trial [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=17664482
191. National Center for Biotechnology Information (NCBI). Brief communication: rituximab in HIV-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=18087054
192. National Center for Biotechnology Information (NCBI). Improved outcome with rituximab in patients with HIV-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=21778341
193. National Center for Biotechnology Information (NCBI). Rituximab decreases the risk of lymphoma in patients with HIV-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22223822
194. National Center for Biotechnology Information (NCBI). Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28143881
195. National Center for Biotechnology Information (NCBI). Remission of HHV-8 and HIV-associated multicentric Castleman disease with ganciclovir treatment [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=14615380
196. National Center for Biotechnology Information (NCBI). Failure of cidofovir in HIV-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=15155471
197. National Center for Biotechnology Information (NCBI). Multicentric Castleman’s disease treated with antivirals and immunosuppressants [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=12966545
198. National Center for Biotechnology Information (NCBI). Tocilizumab in patients with symptomatic Kaposi sarcoma herpesvirus-associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=32276276
199. National Center for Biotechnology Information (NCBI). Tocilizumab in patients with symptomatic Kaposi sarcoma herpesvirus–associated multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7316216
200. National Center for Biotechnology Information (NCBI). High incidence of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=11895764
201. National Center for Biotechnology Information (NCBI). Rituximab therapy for HIV-associated Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=12842986
202. National Center for Biotechnology Information (NCBI). Kaposi Sarcoma Inflammatory Cytokine Syndrome (KICS): A Rare but Potentially Treatable Condition [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28424322
203. National Center for Biotechnology Information (NCBI). Kaposi Sarcoma Inflammatory Cytokine Syndrome (KICS): A Rare but Potentially Treatable Condition [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5423516
204. National Center for Biotechnology Information (NCBI). Surgery in Castleman’s disease: a systematic review of 404 published cases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22367441
205. National Center for Biotechnology Information (NCBI). Impact of siltuximab on patient-related outcomes in multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29391839
206. National Center for Biotechnology Information (NCBI). Impact of siltuximab on patient-related outcomes in multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5769562
207. National Center for Biotechnology Information (NCBI). Patient-reported Outcomes for Multicentric Castleman’s Disease in a Randomized, Placebo-controlled Study of Siltuximab [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=25736164
208. National Center for Biotechnology Information (NCBI). Patient-reported Outcomes for Multicentric Castleman’s Disease in a Randomized, Placebo-controlled Study of Siltuximab [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4383814
209. National Center for Biotechnology Information (NCBI). Deriving health utility values from a randomized, double-blind, placebo-controlled trial of siltuximab in subjects with multicentric Castleman’s disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=26972925
210. National Center for Biotechnology Information (NCBI). Patient reported outcome measures in rare diseases: a narrative review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=29688860
211. National Center for Biotechnology Information (NCBI). Patient reported outcome measures in rare diseases: a narrative review [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5914068
212. National Center for Biotechnology Information (NCBI). The Multicentric Castleman’s Disease (Mcd) -Symptom Scale (Mcd-Ss): Development and Validation of A Patient-Reported Outcome (Pro) Measure for An Ultra-Orphan Disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27201708
213. National Center for Biotechnology Information (NCBI). PDGF receptor mutations in human diseases [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33449152
214. National Center for Biotechnology Information (NCBI). Dysregulated interleukin 6 expression produces a syndrome resembling Castleman’s disease in mice [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=2384605
215. National Center for Biotechnology Information (NCBI). Dysregulated interleukin 6 expression produces a syndrome resembling Castleman’s disease in mice. [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC296765
216. National Center for Biotechnology Information (NCBI). HHV-8-encoded viral IL-6 collaborates with mouse IL-6 in the development of multicentric Castleman disease in mice [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=22490805
217. National Center for Biotechnology Information (NCBI). HHV-8–encoded viral IL-6 collaborates with mouse IL-6 in the development of multicentric Castleman disease in mice [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3370672
218. National Center for Biotechnology Information (NCBI). Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=34265103
219. National Center for Biotechnology Information (NCBI). Multicentric Castleman disease: Where are we now? [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27296355
220. National Center for Biotechnology Information (NCBI). Multicentric Castleman Disease: Where are we now? [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5003700
221. National Center for Biotechnology Information (NCBI). HHV8-related lymphoid proliferations: a broad spectrum of lesions from reactive lymphoid hyperplasia to overt lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=28084335
222. National Center for Biotechnology Information (NCBI). British HIV Association guidelines for HIV-associated malignancies 2014 [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=24528810
223. National Center for Biotechnology Information (NCBI). Human immunodeficiency virus-associated lymphomas in the antiretroviral therapy era: Analysis of the National Cancer Data Base [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27337679
224. National Center for Biotechnology Information (NCBI). Clinical characteristics and survival outcome of primary effusion lymphoma: A review of 105 patients [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=27859456
225. National Center for Biotechnology Information (NCBI). Biology and management of primary effusion lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=30154110
226. National Center for Biotechnology Information (NCBI). Prognostic factors in chemotherapy-treated patients with HIV-associated Plasmablastic lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=20167839
227. National Center for Biotechnology Information (NCBI). Prognostic Factors in Chemotherapy-Treated Patients with HIV-Associated Plasmablastic Lymphoma [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3227958
228. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=Antonino%20Carbone
229. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=Margaret%20Borok
230. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=Blossom%20Damania
231. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=Annunziata%20Gloghini
232. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=Mark%20N.%20Polizzotto
233. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=Raj%20K.%20Jayanthan
234. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=David%20C.%20Fajgenbaum
235. National Center for Biotechnology Information (NCBI). Search Page [Internet]. [cited 2023 Oct 3]. Available from: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&term=Mark%20Bower