Castleman's disease and lymphoma: report of eight cases in HIV-negative patients and literature review

Abstract

Castleman's disease (CD) is a rare atypical lymphoproliferative disorder associated with a risk of developing malignant lymphoma. We have recorded 8 HIV-negative patients presenting this association, 6 with non-Hodgkin's lymphoma (NHL) and 2 with Hodgkin's disease (HD). After literature review, we analyzed all reported cases of association CD-NHL (n = 23) and CD-HD (n = 27). NHL is more often associated with multicentric CD, its diagnosis being concurrent with CD diagnosis or occurring within 2 years. B-NHL is predominant (71%), and mantle cell lymphoma represents 40% of these B-NHL cases. NHL displays an aggressive course and is liable for death, especially in multicentric CD. HD occurs in localized CD of plasma cell type, usually in the same areas, is more often of interfollicular subtype, and its clinical course seems better than NHL. The association of CD and lymphoma seems to be more than coincidental, and its pathogenesis is discussed.