THE SHWACHMAN SYNDROME is a rare autosomal recessive disease associated with exocrine pancreatic insufficiency, skeletal anomalies such as metaphyseal dysostosis, growth retardation, recurrent infections, and hypoplastic hematologic abnormalities, commonly presenting as neutropenia but also with hypoplastic anemia, thrombocytopenia , or pancytopenia. 1-a Recently, several isolated cases of leukemia have been identified in patients with the Shwachman syndrome? -~ Two additional cases of leukemia associated with EPI are described. A common palhophysiologic process in these patients is suggested. CASE REPORTS Patient 1, a 20-month-old boy, presented with diffuse edema, ascites, and failure to thrive. Height and weight were less than the fifth percentile for age. Laboratory studies documented hypoalbuminemia (1.5 gm/dl) with a total protein concentration of 3.4 gm/dl, hemoglobin of 10 gm/dl, white blood count of 14,300/p,1 with normal differential (47% granulocytes), and platelet count of 538,000//d. Urinalysis and sweat chloride determinations were normal. The stools contained markedly increased fat and undigested meat fibers. Duodenal intubation revealed absent trypsin activity in the fluid obtained, and a small bowel biopsy demonstrated subtotal villus atrophy consistent with a malnourished state. The patient was given an elemental diet with resolution of the edema, and subsequently he was advanced to a normal diet with oral pancreatic enzyme and vitamin replacement. Two months later a complete blood count revealed hemoglobin of 13 gm/dl, platelet count 687,000//d, and WBC 11,800/td with an absolute neutrophll count of 2,600/#1. Acute leg pain occurred one month later, when the hemoglobin concentration was 10.4 gm/dl, platelet count 136,000//B, and WBC 33,500/,al with 5% granulocytes and 67% leukemia cells. Bone marrow aspirate and biopsy confirmed the diagnosis of acute lymphocytic leukemia, L-I morphology, with positive periodic acid Schiff staining lymphoblasts. Hematologic remission was successfully induced with vincristine, prednisone, and L-asparaginase. Central nervous system prophylaxis was administered and the patient has