iMCD can mimic the characteristics of malignant, infectious, and autoimmune conditions1
Due to the overlapping symptoms and rarity of the disorder, diagnosing iMCD can take time1,2:
iMCD’s clinical heterogeneity, its lack of specific biomarkers, and its overlapping symptoms with other disorders can make the diagnosis and management of patients with this disease challenging1
Patients experiencing lymphadenopathy with no alternative diagnosis should be evaluated for iMCD with an excisional biopsy1
iMCD may vary from mild constitutional symptoms to a life-threatening cytokine storm3
References: 1. 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. 2. 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. 3. 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. 4. Liu AY, Nabel CS, Finkelman BS, et al. Idiopathic multicentric Castleman's disease: a systematic literature review. Lancet Haematol. 2016;3(4):e163-e175. 5. Mahmud SA, Binstadt BA. Autoantibodies in the pathogenesis, diagnosis, and prognosis of juvenile idiopathic arthritis. Front Immunol. 2019;9:3168. 6. Autoimmune condition. National Cancer Institute. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/autoimmune-condition. Accessed August 15, 2022. 7. Shanbhag S, Ambinder RF. Hodgkin lymphoma: a review and update on recent progress. CA Cancer J Clin. 2018;68(2):116-132. 8. 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. 9. 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. 10. Mononucleosis: symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/mononucleosis/symptoms-causes/syc-20350328. Accessed August 15, 2022. 11. HIV/AIDS: symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/hiv-aids/symptoms-causes/syc-20373524. Accessed August 15, 2022.