Understanding Castleman disease and its subtypes
Castleman disease (CD) describes a group of distinct, nonmalignant lymphoproliferative disorders with a shared histopathology. Classification is based on the number of regions of enlarged lymph nodes and the presence or absence of human herpesvirus-8 (HHV-8).1,2
Classification of CD
Based on lymph node group involvement
Multicentric Castleman Disease (MCD) (~68%)3
Multiple groups of enlarged lymph nodes; subtypes are based on presence or absence of HHV-8 or POEMS syndrome criteria1
A patient diagnosed with human immunodeficiency virus (HIV)–negative and HHV-8–negative MCD is classified as an iMCD patient1
HHV-8–positive MCD (~7.5%)3
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 body4,5
POEMS-associated MCD (~1%)3
POEMS-associated MCD is defined by the presence of either bone lesions or a λ-restricted plasma cell disorder4
Unicentric Castleman Disease (UCD) (~32%)3
A single group of enlarged lymph nodes1
Abbreviations: HIV, human immunodeficiency virus; iMCD, idiopathic multicentric Castleman disease; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
iMCD is a rare lymphoproliferative disorder3,4
iMCD is not a malignancy2
It is characterized by multifocal lymphadenopathy4
It has a poorer prognosis than many malignancies4,6
It is associated with malignancy7
Malignancies arising within the first year of diagnosis (N=119)7
Did you know?
- Every year, approximately 1000 to 1100 patients in the United States are diagnosed with iMCD3
- This rare disease can affect a person regardless of their age, gender, or race8
References: 1. Fajgenbaum DC. Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018;132(22):2323-2330. 2. About Castleman disease. American Cancer Society. https://www.cancer.org/content/dam/CRC/PDF/Public/8594.00.pdf. Revised February 1, 2018. Accessed August 15, 2022. 3. 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. 4. 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. 5. HIV/AIDS: symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/hiv-aids/symptoms-causes/syc-20373524. Accessed August 15, 2022. 6. 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. 7. 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 Annual Meeting and Exposition; December 5-8, 2020; virtual meeting. Oral abstract 2059. 8. Liu AY, Nabel CS, Finkelman BS, et al. Idiopathic multicentric Castleman's disease: a systematic literature review. Lancet Haematol. 2016;3(4):e163-e175.