Castleman’s Disease: A report of Three Cases

Castleman's disease (CD) or angiofollicular lymphoid hyperplasia is a rare lymphoproliferative disorder . It exists in two distinct clinical forms: the localized form is more frequent than the multicentric form which is more rare and aggressive . Histologically, there are three histological types: the hyalino -vascular type, the plasma cell type and the mixed type. The association of this disease with HHV8 infection is strongly suspected. The aim of this work is to study the clinicopathologic features of CD. Six cases of MC were collected in the pathology department of Charles Nicolle hospital in Tunis over a period of 6 years from 2004 to 2010. Our patients were 2 men and 4 women. The mean age was 43.7 years with extremes of 38 and 52 years. 5 patients had localized lymphadenopathy (cervical , retroperitoneal , peripancreatic , inguinal or meso - sigmoid ) . The sixth patient had generalized lymphadenopathy. At histological examination, lymph node parenchyma consisted of nodules delimited by bands of collagen fibrosis. These nodules corresponded to lymphoid follicles with a thick mantle area. Dendritic cells were disposed in concentric circles between which the cells arranged themselves in one layer thereby providing an aspect in “onion bulbs.” They are associated with endothelial hyperplasia. Thus, the diagnosis of localized (n = 5) or multicentric (n = 1) hyalino -vascular type of CD was made. Castleman's disease in its localized form has often a good prognosis. The multicentric form is associated with dys-immune events and has a bad prognosis. The evolution is characterized by the occurrence of infectious complications and the emergence of an aggressive lymphoma.