Castleman disease

Castleman disease (CD), also referred to as angiofollicular lymph node hyperplasia, is a heterogeneous group of lymphoproliferative disorders, characterized by abnormal growth of the lymph nodes. There are three histopathological variants of CD: hyaline-vascular type, which accounts for approximately 80-90% of reported cases; plasma cell type; and an intermediate, or mixed, histological type. Clinical presentation of CD appears to be either localized or generalized (multicentric); however, there is controversy about whether the multicentric form is a distinct entity or simply a form of the plasma cell type (National Organization for Rare Disorders, Inc. [NORD], 2000). There is also some evidence that CD constitutes a spectrum of benign to malignant diseases and that if left untreated, the benign form of the disease may serve as a precursor to the malignant form (Parez et al.).


An occasionally aggressive illness marked by excessive growth of lymphoid tissue either localized in a single lymph node group or in multiple regions of the body. Although the cause is not precisely known, its associations with acquired immunodeficiency syndrome, Kaposi’s sarcoma, and human herpes virus 8 infection have led some experts to propose that it has an infectious basis. Localized disease responds well to surgical resection. Widespread disease can sometimes be treated effectively with chemotherapy.


 


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