MOLECULAR ANALYSIS OF CLONALITY IN CASTLEMANS DISEASE

Castleman's disease (CD) is a rare atypical lymphoproliferative disord er that is morphologically and clinically heterogeneous and is associa ted with a risk of developing malignant lymphoma. We report the clonal ity status of CD tissues in 34 patients, including 14 patients infecte d by the human immunodeficiency virus (HIV). Four patients presented a localized form and 30 presented a multicentric form. Two cases were a ssociated with B-cell lymphoma, 3 cases with Hodgkin's disease, and 9 cases (8 HIV+) with Kaposi's sarcoma. Histologically, 8 cases were of the hyaline-vascular type and 26 were of the plasma cell or mixed type s. The Ig and T-cell receptor (TCR) V(D)J rearrangements were analyzed using polymerase chain reaction and Southern blot. Clonal IgH rearran gements were detected in only 4 cases, ie, 2 associated with B-cell ly mphoma, 1 with Hodgkin's disease, and 1 case without malignancy. A TCR gamma rearrangement of restricted junctional size was amplified in 1 HIV+ case. Finally, polyclonal V-H-J(H) and V-gamma-J(gamma) rearrange ments were detected in the large majority of the cases, irrespective o f pathologic subtypes, clinical forms, and HIV status. The lymphoid co mponent in CD is therefore commonly reactive, and the rare occurrence of detectable monoclonal lymphoid contingents may be caused by seconda ry molecular events. (C) 1995 by The American Society of Hematology.