iMCD’s clinical heterogeneity, as well as its overlap with other disorders and lack of specific
biomarkers, can make it challenging to diagnose and manage patients with iMCD. People
experiencing lymphadenopathy with no alternative diagnosis should be evaluated for iMCD with an
Additionally, tests must be performed to exclude diseases that can mimic iMCD, HHV-8, or human
immunodeficiency virus (HIV) infection.1
Fine-needle aspiration or core biopsy is unlikely to capture the affected tissue within the lymph node and may be inadequate to get an accurate diagnosis of iMCD.2-4
An excisional biopsy enables a pathologist to conduct the complete examination of the total lymph node architecture.2,5
Jadee L. Neff, MD, PhD
If you have a patient who has unexplained lymphadenopathy, unexplained constitutional symptoms, and they can’t seem to get to a diagnosis, I would recommend an excisional biopsy [to] get a complete look at the lymph node.
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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.
Allin D, David S, Jacob A, Mir N, Giles A, Gibbins N. Use of core biopsy in diagnosing cervical lymphadenopathy: a viable alternative to surgical excisional biopsy of lymph nodes? Ann R Coll Surg Engl. 2017;99(3):242-244.
Gaurin GE, da Costa Dourado CM. Castleman disease. Medscape. https://emedicine.medscape.com/article/2219018-overview. Updated November 24, 2021. Accessed April 21, 2022.
Early detection, diagnosis, and staging of Castleman disease. American Cancer Society. https://www.cancer.org/content/dam/CRC/PDF/Public/8596.00.pdf. Revised February 8, 2018. Accessed May 11, 2022.
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.
Mahmud SA, Binstadt BA. Autoantibodies in the pathogenesis, diagnosis, and prognosis of juvenile idiopathic arthritis. Front Immunol. 2019;9:3168.
Autoimmune condition. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/autoimmune-condition. Accessed June 9, 2022.
Shanbhag S, Ambinder RF. Hodgkin lymphoma: a review and update on recent progress. CA Cancer J Clin. 2018;68(2):116-132.
Cheson BD, Fisher RI, Barrington SF, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059-3067.
Fugl A, Andersen CL. Epstein-Barr virus and its association with disease - a review of relevance to general practice. BMC Fam Pract. 2019;20(1):62.
Mononucleosis: symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/mononucleosis/symptoms-causes/syc-20350328. Published December 22, 2020. Accessed June 9, 2022.
HIV/AIDS: symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/hiv-aids/symptoms-causes/syc-20373524. Published February 13, 2020. 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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