CENTRAL-NERVOUS-SYSTEM INVOLVEMENT AT DIAGNOSIS IN A CASE OF PEDIATRIC CD30-CELL LYMPHOMA( ANAPLASTIC LARGE)

Central nervous system (CNS) involvement in Ki-1/CD30 lymphoma is extr emely rare, in contrast to the frequent involvement in other types of pediatric non-Hodgkin's lymphoma. No mechanism has yet been proposed t o explain the sparing of the blood brain barrier in Ki-1/lymphoma. We present a 2-year-old boy who was admitted to the Department of Pediatr ic Hemato-Oncology due to lethargy progressive breathing difficulties, massive diffuse lymphadenopathy, hepatosplenomegaly, and ichthyosis-l ike skin involvement with epidermolysis. A lymph node biopsy was compa tible with Ki-1/CD30 anaplastic large cell lymphoma (ALCL). Bone marro w aspirate and biopsy demonstrated reactive hyperplasia. Cytogenetic a nalysis displayed hyperdiploid cells with 1p(-) in most cells. Cerebro spinal fluid examination showed pleocylosis with CD30+ cells. Possible mechanisms which could enable CNS involvement in this unusual case ar e discussed. (C) 1997 Wiley-Liss, Inc.