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SynopsisCastleman disease (CD), or angiofollicular lymph node hyperplasia, is a rare non-clonal lymphoproliferation that may be associated with human immunodeficiency virus (HIV) and human herpesvirus 8 (HHV-8) and several malignancies including Kaposi sarcoma (KS), non-Hodgkin lymphoma, Hodgkin lymphoma, and POEMS syndrome (a syndrome of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma, and skin changes and sclerotic bone changes).
There are 2 subsets of the disease, unicentric (UCD) and multicentric (MCD):
- UCD is characterized by an asymptomatic isolated benign lymphoproliferation occurring in patients with a mean age of 35 and is not associated with HHV-8 infection. UCD is commonly discovered incidentally on imaging studies and is curable with surgical resection.
- MCD is a systemic disease that presents later in life (median age, between 52 and 65) and is associated with immunosuppression and HHV-8 infection. It is characterized by a generalized peripheral lymphadenopathy and is commonly associated with hepatosplenomegaly, fevers, weight loss, weakness / fatigue, and night sweats. Most patients with MCD will die from fulminate infections, progressive disease, or related malignancies including Kaposi sarcoma, non-Hodgkin lymphoma, Hodgkin lymphoma, and POEMS syndrome.
Related topics: HIV, HHV-8, Kaposi Sarcoma, Non-Hodgkin Lymphoma, Hodgkin Lymphoma, POEMS Syndrome
D47.Z2 – Castleman disease
207036003 – Castleman's Disease
Differential Diagnosis & PitfallsCD is rare, and more common causes of lymphadenopathy should be considered. These are numerous and include infectious etiologies, malignancies, lymphoproliferative diseases, immune-mediated disorders, and drug reactions.