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儿童原发性(特发性)血小板增多症一例的形态学与功能研究(作者译)

[Morphological and functional studies in a case of primary (idiopathic) thrombocythaemia in childhood (author's transl)].

作者信息

Wündisch G F, Steidle C, Balleisen L, Ströder W

出版信息

Klin Wochenschr. 1977 Oct 15;55(20):995-1003. doi: 10.1007/BF01488186.

Abstract

Primary (idiopathic) thrombocythaemia is a rare disease in childhood. Haemorrhagic diathesis and splenomegaly are principal findings besides thrombocytosis. The cases of a nine year old girl with thrombocyte levels between 2.8 and 4.5 X 10(6) microliter is described. The examination in light and electron microscopy revealed various morphological abnormalities in thrombocytes and megakaryocytes. Platelet function was disturbed in various degrees. The impact of functional abnormalities on the haemorrhagic diathesis is discussed. The findings in this child were compared to those of the better characterized picture of primary and secondary thrombozytosis in adulthood. Possible pathogenic relations to myeloproliferative diseases are also discussed.

摘要

原发性(特发性)血小板增多症在儿童时期是一种罕见疾病。除血小板增多外,出血素质和脾肿大是主要表现。本文描述了一名9岁女孩的病例,其血小板水平在2.8至4.5×10⁶/微升之间。光镜和电镜检查显示血小板和巨核细胞存在各种形态异常。血小板功能受到不同程度的干扰。文中讨论了功能异常对出血素质的影响。将该患儿的检查结果与成年期原发性和继发性血小板增多症特征更明显的情况进行了比较。还讨论了与骨髓增殖性疾病可能的致病关系。

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本文引用的文献

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