Central nervous system involvement at diagnosis in a case of pediatric CD30+ anaplastic large cell lymphoma

Central nervous system (CNS) involvement in Ki‐1/CD30 lymphoma is extremely rare, in contrast to the frequent involvement in other types of pediatric non‐Hodgkin's lymphoma. No mechanism has yet been proposed to 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 Pediatric Hemato‐Oncology due to lethargy, progressive breathing difficulties, massive diffuse lymphadenopathy, hepatosplenomegaly, and ichthyosis‐like skin involvement with epidermolysis. A lymph node biopsy was compatible with Ki‐1/CD30 anaplastic large cell lymphoma (ALCL). Bone marrow aspirate and biopsy demonstrated reactive hyperplasia. Cytogenetic analysis displayed hyperdiploid cells with 1p(−) in most cells. Cerebrospinal fluid examination showed pleocytosis with CD30+ cells. Possible mechanisms which could enable CNS involvement in this unusual case are discussed. Med. Pediatr. Oncol. 28:132–135 © 1997 Wiley‐Liss, Inc.