A Rare Lymphoproliferative Disease: Castleman Disease.

Castleman disease, is a rare lymphoproliferative disease, also known as angiofollicular lymph node hyperplasia, It is classified as hyaline vascular and plasmacytic variant histologically but the characteristics of both types can coexist. Most of unicentric disease is hyalin vascular while most of multicentric disease is plasmacytic type. Although the pathogenesis is not completely understood, the role of IL-6 in unicentric disease and the role of IL-6 and HHV-8 in multicentric disease is well defined. Unicentric disease is typically localized, symptoms are minimal and treated locally. Multicentric disease is a systemic disease and is clinically characterized with generalized lymphadenopathy, splenomegaly, anemia and systemic inflammatory symptoms. Systemic therapies are given primarily. Several malignant diseases (lymphomas, POEMS syndrome, follicular dendritic cell sarcomas, paraneoplastic pemphigus, Kaposi sarcoma, amyloidosis) can be associated with Castleman disease. In this paper; recent information about Castleman disease, which is a rare disease, is summarized.