Castleman Disease

**Semantic type:** Disease or Syndrome

**Definition:** A disorder characterized by lymphoid hyperplasia in the lymph nodes. There are two histologic variants recognized: the hyaline-vascular type and the plasma cell type. In the hyaline vascular type there are hyalinized vessels present in the lymphoid follicles. In the plasma cell type there is pronounced plasma cell proliferation. Clinically, the disease may be localized; manifested with localized lymphadenopathy, or multicentric; manifested with generalized lymphadenopathy, fever, organomegaly, and sometimes POEMS syndrome. In contrast to patients with localized disease who are usually cured following resection of the lesion, patients with the multicentric form of the disease may follow a progressive clinical course, complicated by infection, Kaposi sarcoma or lymphoma.

**Synonyms:** - AFLH - Angiofollicular Lymphoid Hyperplasia - Castleman's Disease - Castleman's Tumor - Castleman's disease - GLNH - Giant Lymph Node Hyperplasia