PERIPHERAL T-CELL LYMPHOMA IN CHILDHOOD - A REPORT OF 5 CASES IN TAIWAN

We encountered five children with peripheral T-cell lymphoma (PTL) at National Taiwan University Hospital (NTUH) from 1985-1989. The patient s were four boys and one girl, aged between 5 and 13 years. The durati on of prediagnostic symptoms varied from 1 month to 5 years. All had p yrexia and lymphadenopathy; one had a prolonged history of granulomato sis with repeated infection. Four had hepatosplenomegaly. One patient presented with diffuse pulmonary infiltration and impending respirator y failure. All patients were negative for human T-cell leukemia virus (HTLV)-I antibody, and positive for HBsAg. Four patients who had EBV-v iral capsid antigen (VCA) IgG and who were IgM tested were positive fo r EBV-VCA IgG, but only two had evidence of active EBV infection. Tumo r cell markers were examined and showed the following phenotypes: all patients were CD2, CD3, and CD7 positive but CD19 and CD20 negative; t hree patients were CD4 positive and CD8 negative; the other two patien ts were CD4 negative and CD8 positive. Four patients died 2-7 months a fter diagnosis. The remaining patient received allogeneic bone marrow transplantation and has survived free of disease for more than 22 mont hs after transplant. Our five cases reconfirm the high frequency of di agnostic delay, the heterogenous immunophenotypes, high mortality, and poor responsiveness to conventional therapy for PTL. Bone marrow tran splantation in the early stage might be a possible cure of this diseas e. (C) 1994 Wiley-Liss, Inc.