ACUTE-LEUKEMIA COMPLICATING BONE-MARROW HYPOPLASIA IN AN ADULT WITH SHWACHMAN-SYNDROME

被引：23

作者：

SEYMOUR, JF ^{[1
]}

ESCUDIER, SM ^{[1
]}

机构：

[1] UNIV TEXAS,MD ANDERSON CANC CTR,DEPT HEMATOL,LEUKEMIA SECT,1515 HOLCOMBE BLVD,HOUSTON,TX 77030

来源：

LEUKEMIA & LYMPHOMA | 1993年 / 12卷 / 1-2期

关键词：

SHWACHMAN-SYNDROME; ACUTE LEUKEMIA; APLASTIC ANEMIA;

D O I：

10.3109/10428199309059581

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页码：131 / &

共 26 条

[1] Shwachman H., Diamond L.K., Oski F.A., Khaw K.T., The syndrome of pancreatic insufficiency and bone marrow dysfunction, J. Pediatr., 65, pp. 645-663, (1964)
[2] Shmerling D.H., Prader A., Hitzig W.H., Giedion A., Hadorn Z.B., Kuhni M., The syndrome of exocrine pancreatic insufficiency, neutropenia, metaphyseal dysostosis and dwarfism, Helvet. Pcediatr. Acta, 24, pp. 547-575, (1969)
[3] Burke V., Colebatch J.H., Anderson C.M., Simons M.J., Association of pancreatic insufficiency and chronic neutropenia in childhood, Arch. Diseases Childhood, 42, pp. 147-157, (1967)
[4] Alter B.P., Fanconi's anemia. Current concepts, Am. J. Pediatr. Hematol./Oncol., 14, pp. 170-176, (1992)
[5] Aggett P.J., Cavanagh N.P.C., Matthew D.J., Pincott J.R., Sutcliffe J., Harries J.T., Shwachman's syndrome. A review of 21 cases, Arch. Diseases Childhood, 55, pp. 331-347, (1980)
[6] Alter B.P., The bone marrow failure syndromes, Hematology of Infancy and Childhood, pp. 159-234, (1987)
[7] Marsh J.C.W., Geary C.G., Annotation. Is aplastic anemia a pre-leukemic disorder, Br. J. Haematol., 77, pp. 447-452, (1991)
[8] Marsh J.C.W., Will A.J., Hows J.M., Stem cell” origin of the hematopoietic defect in dyskeratosis congenita, Blood, 79, pp. 3138-3144, (1992)
[9] Young N.S., The problem of clonality in aplastic anemia: Dr. Dameshek's riddle, restated, Blood, 79, pp. 1385-1392, (1992)
[10] Lawrence H.J., Largman C., Homeobox genes and normal hematopoiesis and leukemia, Blood, 80, pp. 2445-2453, (1992)

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