Acute leukemia complicating bone marrow hypoplasia in an adult with Shwachman's syndrome

Leukemia & Lymphoma
J F Seymour, S M Escudier

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.

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Citations

Dec 28, 2011·Musculoskeletal Surgery·C Dall'ocaP Bartolozzi
Aug 16, 2002·British Journal of Haematology·Yigal Dror, Melvin H Freedman
Dec 11, 2002·British Journal of Haematology·Joan CunninghamNorman Pratt
Apr 17, 2002·Seminars in Hematology·O P Smith
Mar 10, 2004·International Journal of Hematology·Tetsuo MitsuiKiyoshi Hayasaka
Apr 12, 2012·Haematologica·Jean DonadieuUNKNOWN Associated investigators of the French Severe Chronic Neutropenia Registry*
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Aug 31, 2002·Bone Marrow Transplantation·J W HsuR A Brodsky
Jul 28, 2005·Pediatric Blood & Cancer·Yigal Dror
Jul 11, 2006·Seminars in Hematology·Akiko Shimamura

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