Castleman 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.
CD may be associated with paraneoplastic pemphigus that can be the initial presenting symptom in both variants. CD is the most common neoplasm associated with paraneoplastic pemphigus in adolescents and children and the third most common in adults, following non-Hodgkin lymphoma and chronic lymphocytic leukemia.
CD 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.
