FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS ASSOCIATED WITH DISSEMINATED T-CELL LYMPHOMA - A REPORT OF 2 SIBLINGS

Two siblings with evidence of disseminated T-cell lymphoma at the time of diagnosis of familial hemophagocytic lymphohistiocytosis (FHL) are reported, an association which has not been described previously. The first child with typical clinical and laboratory features of FHL died shortly after admission, before diagnosis could be established. Retro spective analysis of autoptic tissue revealed marked hemophagocytosis as well as morphological and immunohistochemical features suggestive o f disseminated T-cell lymphoma. In the second child, FHL was diagnosed in time. Subsequent histologic investigation of bone marrow biopsies displayed a focal infiltration by T-cell lymphoma. DNA hybridization s tudies provided evidence of a monoclonal T-cell receptor beta chain ge ne rearrangement. Following conventional chemotherapeutic induction fo r FHL, the patient received an allogeneic bone marrow transplant (BMT) from a related healthy donor. Currently, 17 months after BMT, the boy is in unmaintained remission from FHL and T-cell lymphoma. The curren t pathogenetic concepts for FHL and a possible relationship between T- cell lymphoma and FHL are discussed.