OBJECTIVETo study the pathogenesis and the differential diagnosis of Castleman's disease.

METHODSHistopathology, immunohistochemical staining and clinical courses of 26 cases of Castleman's disease (CD) were studied with follow-up study of 16 cases.

RESULTSThe present study included 6 cases of multicentric type, 20 cases of localized type in the clinical aspects and 19 cases with hyaline vascular type, 4 cases with plasma cell type, 3 cases with mixed type in the histologic aspect. The Multicentric type presented systemic lymphadenopathy, anemia, hyperglobulinemia, hepatosplenomegaly, skin changes, and lung disorder and kidney disfunction, of which 1 case died of respiratory and renal insufficiency. 13 of the 20 localized cases were of the hyaline vascular type, and with good prognosis. 7 of the 20 cases showed paraneoplastic pemphigus associated with hyperglobulinemia (4/7) and lung disease (5/7). The pathologic features composed of proliferation of the mantle zone B cell, follicular dendritic cell, plasma cell and small vessels. In immunohistochemical staining, kappa and lambda light chains were detected in each CD case.

CONCLUSIONSMany diseases are similar to CD clinicopathologically. It is important to make differential diagnosis through pathological study. Castleman's disease is a lymphoproliferative disorder. The pathogenesis of this multicentric disorder may be associated with autoimmune disease.