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C27855Level 9

Multicentric Castleman Disease

**Semantic type:** Neoplastic Process

**Definition:** Castleman disease characterized by fever, generalized lymphadenopathy, hypergammaglobulinemia, and dysfunction of multiple organs. Other signs and symptoms include anemia, thrombocytopenia, hepatomegaly, peripheral neuropathy and pleural effusions. Morphologically, in the majority of cases the lymph nodes show features of Castleman disease of the plasma cell type. In a minority of cases, changes of Castleman disease of the hyaline-vascular type are seen. 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:** - Idiopathic Multicentric Castleman Disease - MCD - Multicentric Angiofollicular Lymphoid Hyperplasia - Multicentric Angiofollicular Lymphoid Hyperplasia - Multicentric Castleman's Disease - iMCD

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