Primary cutaneous T-cell-rich B-cell lymphoma and Hodgkin's disease in a patient with Gardner's syndrome

A 50-year-old patient, suffering from familial polyposis (Gardner's syndrom e), initially presented with several nodules on his left arm. Histological examination revealed primary cutaneous T-cell-rich B-cell lymphoma (TCRBCL) . Staging procedures failed to detect any systemic involvement. Three years after total excision of the tumours, the patient presented with a non-spec ific dermatitis, enlarged axillary lymph nodes and splenomegaly. Histologic al and immunohistochemical examination of lymph node and spleen biopsy spec imens resulted in the diagnosis of Hodgkin's disease (HD) of the nodular sc lerosis type. Sequence analysis of single cells micromanipulated from skin and from lymph node lesions indicated that both lymphoma infiltrates were d erived from the same precursor germinal centre B-cell clone. This is a case showing a clonal relationship between TCRBCL and HD, providing support to the B-cell origin of Hodgkin and Reed-Sternberg cells. Copyright (C) 2000 S . Karger AG, Basel.