iMCD: A mysterious condition that mimics many other diseases
About iMCD
Lymphadenopathy with an inconclusive diagnosis? Consider iMCD.
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 (iMCD).
What is iMCD?
iMCD is a chronic and progressive lymphoproliferative disorder of ≥2 lymph node groups throughout the body.1 People who have iMCD are HIV negative and HHV-8 negative.2
If your patient has tested negative for malignant, infectious, and autoimmune diseases, you should consider iMCD.1
iMCD: A rare lymphoproliferative disorder1,3
- iMCD is not a malignancy4
- It is characterized by multifocal lymphadenopathy1
- It has a poorer prognosis than many malignancies1,5
- It is associated with malignancy6
About 1000 to 1100 new patients are diagnosed with iMCD in the United States every year3
100% of patients with iMCD have lymphadenopathy7
Malignancies arising within the first year after diagnosis (N=119)6
Understanding Castleman disease and its subtypes
Castleman disease (CD) describes a group of distinct, nonmalignant lymphoproliferative disorders with a shared histopathology.2,4
Multicentric Castleman Disease (MCD)
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
Unicentric Castleman Disease (UCD)
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
iMCD: Likely the most prevalent subtype of Castleman disease
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 criteria.1,2,4,8,9
iMCD
A patient diagnosed with human immunodeficiency virus (HIV)–negative and HHV-8–negative MCD is classified as an iMCD patient.2
POEMS-associated MCD
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
HHV-8–positive MCD
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, MPHThe diagnosis of iMCD could be extremely challenging, and one initially has to rule out some of these other mimicking conditions.
Common symptoms of iMCD
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:
Generalized Lymphadenopathy1,7
Generalized Lymphadenopathy1,7
Enlargement seen across multiple groups of lymph nodes
100% of patients with iMCD have lymphadenopathy7
Flu-like Symptoms1,7
Flu-like Symptoms1,7
Fevers, night sweats, fatigue, and weight loss14
89% of patients with iMCD have flu-like symptoms3
Fluid Accumulation1,7
Fluid Accumulation1,7
Edema, ascites, and/or other symptoms of fluid overload
58% of patients with iMCD have fluid accumulation3
Organomegaly1,7
Organomegaly1,7
Enlarged liver or spleen
24% of patients with iMCD have organomegaly3
In patients with iMCD, interleukin-6 (IL-6) levels may be elevated during flare-ups and correlate with the intensity of their symptoms2,7,10
Overproduction of IL-6 is a common pathological driver of iMCD11
IL-6 plays an important homeostatic role as part of the body’s immune system by regulating B cells and T cells12
IL-6 is a cytokine that is typically produced because of an infection or tissue damage12
IL-6 triggers B cells to produce antibodies in the germinal centers within the lymph nodes13
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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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Your report
A diagnosis of idiopathic multicentric Castleman disease (iMCD) requires the presence of multiple swollen or enlarged lymph nodes. In some patients, the swollen lymph nodes may not be visible and may require imaging tests to confirm their presence. These symptoms alone are not enough to diagnose iMCD, as they could be signs of other diseases.
Talk to your doctor if you have any questions about this report.
Additional information about iMCD is available for healthcare professionals at KnowiMCD.com/HCP.
To protect your privacy, KnowiMCD.com does not save any of your information. Please be sure to download, save, and/or print this report.
Healthcare professionals can find information about the latest diagnostic criteria and treatment options by visiting
Your report
A diagnosis of idiopathic multicentric Castleman disease (iMCD) requires the presence of multiple swollen or enlarged lymph nodes. In some patients, the swollen lymph nodes may not be visible and may require imaging tests to confirm their presence. These symptoms alone are not enough to diagnose iMCD, as they could be signs of other diseases.
Talk to your doctor if you have any questions about this report.
Additional information about iMCD is available for healthcare professionals at KnowiMCD.com/HCP.
To protect your privacy, KnowiMCD.com does not save any of your information. Please be sure to download, save, and/or print this report.
Possible tests
In order to confirm a diagnosis of iMCD, a doctor will need to perform tests to detect the presence of multiple swollen lymph nodes.
- Physical examination
- Lab tests—blood or urine test
- Imaging tests—CT scan, x-ray, MRI, PET scan
- Excisional biopsy
By performing some of the above-mentioned tests, a doctor will be able to eliminate the possibility of autoimmune, cancerous, and infectious diseases. An excisional biopsy of the enlarged lymph node can confirm the diagnosis of iMCD.
The road to diagnosis
iMCD is a rare disease that can be difficult to diagnose because it can mimic so many other diseases. It may take a while to receive an accurate diagnosis because your doctor must rule out several different conditions before suspecting iMCD.
You may start your diagnosis journey at your primary care physician’s office, where you may be referred to a specialist.
Preparing for your appointment
Here are some tips to help you have a conversation with your doctor:
- Write down any symptoms you’ve experienced, even if they may seem unrelated
- Share the results of this Symptom Checker with your doctor
By performing some of the above-mentioned tests, a doctor will be able to eliminate the possibility of autoimmune, cancerous, and infectious diseases. An excisional biopsy of the enlarged lymph node can confirm the diagnosis of iMCD.
Start the conversation
Here are some tips to help you talk to your doctor about iMCD:
- What kinds of tests might I need to confirm iMCD?
- Do I need to plan for follow-up visits?
- What treatments are available for iMCD? How will they affect my life?
- Do you have any educational materials that I can take with me?
- What else do I need to know about iMCD?
Notes:
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US-NPS-2200024 1/22
Healthcare professionals can find information about the latest diagnostic criteria and treatment options by visiting
Your iMCD Diary
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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 diagnosis.
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 managing infections.
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.
Hemophagocytic lymphohistiocytosis.
Human immunodeficiency virus.
Human herpesvirus-8.
A doctor who specializes in the branch of medical science that deals with cancers of the blood and blood-forming tissues.
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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