title: Acute leukemia complicating bone marrow hypoplasia in an adult with Shwachman's syndrome.
abstract: Shwachman's syndrome is a rare congenital disorder associated with neutropenia and exocrine pancreatic insufficiency. We describe the development of acute myeloid leukemia in a 38-year-old patient with Shwachman's syndrome following three years of pancytopenia. After chemotherapy the leukemic clone was eradicated, however, the patient's bone-marrow hypoplasia persisted beyond 180 days with neutropenia that responded to administration of granulocyte colony-stimulating factor. Despite the patient's low erythropoietin levels, administration of erythropoietin did not improve his hemoglobin. We review previously reported cases of leukemia complicating Shwachman's syndrome with emphasis on the persistent risk of complications in patients with congenital bone-marrow failure syndromes.
authors.affiliation: Department of Medical Oncology, University of Texas, M. D. Anderson Cancer Center, Houston 77030.
authors.forename: J F
authors.initials: JF
authors.lastname: Seymour
authors.forename: S M
authors.initials: SM
authors.lastname: Escudier
mesh_terms: D000208:Acute Disease; D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001853:Bone Marrow / Q000473:pathology*; D003561:Cytarabine / Q000008:administration & dosage; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D007951:Leukemia, Myeloid / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008297:Male; D009503:Neutropenia / Q000150:complications / Q000473:pathology*; D010182:Pancreatic Diseases / Q000150:complications / Q000473:pathology*; D013577:Syndrome; D014740:Vidarabine / Q000008:administration & dosage / Q000031:analogs & derivatives
chemical_list: D000970:Antineoplastic Agents; D003561:Cytarabine; D016179:Granulocyte Colony-Stimulating Factor; D014740:Vidarabine; C024352:fludarabine