PRIMARY HEPATIC NON-HODGKINS-LYMPHOMA IN CHILDREN - A CASE-REPORT ANDREVIEW OF THE LITERATURE

Non-Hodgkin's lymphomas presenting exclusively in the liver are rather uncommon in adults and extremely rare in children. We describe a six- year-old white boy with jaundice, abdominal pain, and weight loss of t wo weeks duration. Physical examination disclosed asthenia, jaundice, abdominal swelling, large hepatomegaly, and ascitis. Aminotransferases , bilirubin, and alkaline phosphatase were significantly elevated. Bon e marrow aspiration, cerebrospinal fluid, chest x-ray, renal function tests, and uric acid were normal. Abdominal ultrasound showed liver en largement with irregular borders, many parenchymal nodules in both liv er robes, a large hypoechogenic mass in the inferior segment of the li ver, normal biliary ducts and two pancreatic nodules resembling those in the liver. Liver needle biopsy disclosed diffuse lymphomatous infil tration. Blast cells were positive for leukocyte common antigen (CD 45 ). Immunohistochemistry study for T or B cell lineage differentiation was not done. The child showed an excellent response to chemotherapy b ased on the BFM-83 protocol for B cell non-Hodgkin's lymphomas. The pa tient had his therapy discontinued in lune 1995 and remains in first c omplete remission as of May 20th, 1996. (C) 1997 Wiley-Liss, Inc.