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Shwachman's syndrome and leukaemia.

作者信息

Caselitz J, Klöppel G, Delling G, Grüttner R, Holdhoff U, Stern M

出版信息

Virchows Arch A Pathol Anat Histol. 1979 Dec;385(1):109-16. doi: 10.1007/BF00433546.

DOI:10.1007/BF00433546
PMID:162096
Abstract

The clinical and morphological characteristics of Shwachman's syndrome (exocrine pancreatic insufficiency, pancytopenia, skeletal changes) were observed in a boy who, at the age of 8 years, developed a juvenile form of chronic myeloic leukemia which did not respond to cytostatic treatment. Autopsy revealed a striking lipomatous atrophy of the pancreas, defects in the ossification zones of the bones and marked dwarfism. In addition there was leukaemic infiltration of the pancreas, the spleen, the liver and the lymph nodes. The association of Shwachman's syndrome with leukaemia is a rare, but remarkable complication of this entity because of its relationship to the preceeding pancytopenia. Thorough follow-up of the haematological status of patients with Shwachman's syndrome is recommended.

摘要

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本文引用的文献

1
[Lipomatous cystic pancreas fibrosis and lipmatous pancreas atrophy in childhood].儿童脂肪性胰腺囊肿纤维化及脂肪性胰腺萎缩
Beitr Pathol Anat. 1959;121:64-80.
2
THE SYNDROME OF PANCREATIC INSUFFICIENCY AND BONE MARROW DYSFUNCTION.胰腺功能不全与骨髓功能障碍综合征
J Pediatr. 1964 Nov;65:645-63. doi: 10.1016/s0022-3476(64)80150-5.
3
CONGENITAL HYPOPLASIA OF THE EXOCRINE PANCREAS.先天性外分泌胰腺发育不全
Acta Paediatr (Stockh). 1964 May;53:282-93. doi: 10.1111/j.1651-2227.1964.tb07237.x.
4
Association of pancreatic insufficiency and chronic neutropenia in childhood.
Arch Dis Child. 1967 Apr;42(222):147-57. doi: 10.1136/adc.42.222.147.
5
[Metaphyseal dysostosis and congenital pancreatic insufficiency].
Fortschr Geb Rontgenstr Nuklearmed. 1968 Jan;108(1):51-7.
6
The syndrome of exocrine pancreatic insufficiency, neutropenia, metaphyseal dysostosis and dwarfism.外分泌性胰腺功能不全、中性粒细胞减少、干骺端发育不良和侏儒症综合征。
Helv Paediatr Acta. 1969 Dec;24(6):547-75.
7
[Exocrine pancreatic insufficiency, neutropenia, metaphyseal dysostosis and retarded growth (Shwachman's syndrome)].
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8
Metaphyseal dysostosis and the associated syndrome of pancreatic insufficiency and blood disorders.
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9
Preleukemia. The hematologic syndrome preceding acute leukemia.
Am J Med. 1973 Jul;55(1):38-48. doi: 10.1016/0002-9343(73)90148-4.
10
[Exocrine pancreatic insufficiency, neutropenia, metaphyseal dysostosis and growth retardation (Shwachman syndrome) with transient disturbances of carbohydrate metabolism].
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