If you suspect that your patient might have lymphoma or myeloma but are unable to find a
conclusive diagnosis, you may want to consider idiopathic multicentric Castleman disease
About 1000 to 1100 new patients are diagnosed with iMCD in the United States every year3
Malignancies arising within the first year after diagnosis (N=119)6
Castleman disease (CD) describes a group of distinct, nonmalignant lymphoproliferative disorders with a shared histopathology.2,4
MCD involves multiple groups of enlarged lymph nodes and may require ongoing treatment to keep the symptoms under control.2,4
~68% of all CD cases3
UCD involves a single group of enlarged lymph nodes and can be treated by surgically removing the affected lymph node.2,4
~32% of all CD cases3
There are 3 known subtypes of MCD. Their classifications are based on the number of
regions of enlarged lymph nodes and the presence or absence of human herpesvirus-8 (HHV-8) or
the POEMS syndrome
A patient diagnosed with human immunodeficiency virus (HIV)–negative and HHV-8–negative MCD is
classified as an iMCD patient.2
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes
(POEMS)–associated MCD is defined by the presence of either bone lesions or a λ-restricted
plasma cell disorder.1
This subtype of iMCD can cause Kaposi’s sarcoma—a rare cancer in which lesions grow on the skin,
mouth, nose, throat, and other parts of the body.1,4,8,9
iMCD is a rare disease that can be difficult to diagnose, as its characteristics are similar
to cancers such as non-Hodgkin lymphoma, autoimmune disorders such as rheumatoid arthritis, and
infectious disorders such as HIV.1
Corey Casper, MD, MPH
The diagnosis of iMCD could be extremely challenging, and one initially has to rule out some of these other mimicking conditions.
The presentation of iMCD can vary from patient to patient. It can range from mild constitutional symptoms such as fever and night sweats to a life-threatening cytokine storm, which can lead to organ failure.3,5,7
Here are some of the most commonly observed symptoms:
Enlargement seen across multiple groups of lymph nodes
100% of patients with iMCD have lymphadenopathy7
Fevers, night sweats, fatigue, and weight loss14
89% of patients with iMCD have flu-like symptoms3
Edema, ascites, and/or other symptoms of fluid overload
58% of patients with iMCD have fluid accumulation3
Enlarged liver or spleen
24% of patients with iMCD have organomegaly3
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Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-1657.
Fajgenbaum DC. Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018;132(22):2323-2330.
Mukherjee S, Martin R, Sande B, Paige JS, Fajgenbaum DC. Epidemiology and treatment patterns of idiopathic multicentric Castleman disease in the era of IL-6-directed therapy. Blood Adv. 2022;6(2):359-367.
About Castleman disease. American Cancer Society. https://www.cancer.org/content/dam/CRC/PDF/Public/8594.00.pdf. Revised February 1, 2018. Accessed May 11, 2022.
Sitenga J, Aird G, Ahmed A, Silberstein PT. Impact of siltuximab on patient-related outcomes in multicentric Castleman’s disease. Patient Relat Outcome Meas. 2018;9:35-41.
Mukherjee S, Martin R, Sande B, et al. A longitudinal population level analysis of healthcare resource utilization, comorbidity, and survival in idiopathic multicentric Castleman disease patients. Presented at: 62nd American Society of Hematology (ASH) Annual Meeting and Exposition; December 5-8, 2020; virtual meeting.
Liu AY, Nabel CS, Finkelman BS, et al. Idiopathic multicentric Castleman’s disease: a systematic literature review. Lancet Haematol. 2016:3(4):e163-e175.
Dictionary of Cancer Terms: kaposi sarcoma. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/kaposi-sarcoma. Accessed June 9, 2022.
Dictionary of Cancer Terms: kaposi sarcoma-associated herpesvirus. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/kaposi-sarcoma-associated-herpesvirus. Accessed June 9, 2022.
England JT, Abdulla A, Biggs CM, et al. Weathering the COVID-19 storm: lessons from hematologic cytokine syndromes. Blood Rev. 2021;45:100707.
van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018;132(20):2115-2124.
Garbers C, Heink S, Korn T, Rose-John S. Interleukin-6: designing specific therapeutics for a complex cytokine. Nat Rev Drug Discov. 2018;17(6):395-412.
Choy EH, De Benedetti F, Takeuchi T, Hashizume M, John MR, Kishimoto T. Translating IL-6 biology into effective treatments. Nat Rev Rheumatol. 2020;16(6):335-345.
Dictionary of Cancer Terms: flu-like symptoms. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/flu-like-symptoms. Accessed June 9, 2022.
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Viral hemophagocytic lymphohistiocytosis.
A doctor who specializes in the branch of medicine that deals with rheumatic conditions.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
Positron emission tomography (PET) is a method of producing a 3-dimensional image of the internal
structures of body tissues after administering a natural biochemical substance like glucose or fatty
acids. These images are generated with the help of a computer.
A doctor who evaluates the changes caused by disease in tissues and body fluids and helps reach a
A doctor who specializes in the diagnosis and treatment of kidney abnormalities.
Macrophage activation syndrome.
Malignancy-associated hemophagocytic lymphohistiocytosis.
A disease in which cells of the lymph system grow excessively
Lymph nodes make immune cells that help the body fight infection.
A key signaling molecule that helps to regulate the body’s immune system.
Infectious disease medicine is the subspecialty of internal medicine that focuses on diagnosing and
A rare condition that affects multiple groups of lymph nodes throughout the body. Patients with iMCD
are HIV negative and HHV-8 negative.
The underlying cause or origin of a disease is unknown.
Human immunodeficiency virus.
A doctor who specializes in the branch of medical science that deals with cancers of the blood and
Powerful molecules that are normally secreted by the cells of the immune system in response to
infectious or noninfectious agents.
An overreaction of the body’s immune system in which too many cytokines are released into the
bloodstream very quickly, with potentially severe or life-threatening effects.
Computed tomography (CT) is a method of producing an image of the internal structures by passing an
x‑ray through the body.
The removal and examination of tissue, cells, or fluids from the body.
A condition in which the body’s immune system accidentally attacks itself.
Autoimmune lymphoproliferative syndrome.
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