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血栓性血小板减少性紫癜、溶血尿毒综合征与弥散性血管内凝血的比较免疫病理学研究

Comparative immunopathologic studies of thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome and disseminated intravascular coagulation.

作者信息

Hayasaka T, Yoshiki T, Shirai T, Ninomura N, Itoh T

出版信息

Acta Pathol Jpn. 1981 Jul;31(4):569-81. doi: 10.1111/j.1440-1827.1981.tb02754.x.

DOI:10.1111/j.1440-1827.1981.tb02754.x
PMID:7197110
Abstract

The vascular lesion of thrombotic thrombocytopenic purpura was characterized by two distinct types of changes in the arteriole of the heart and brain: (1) multiple incomplete vascular occlusions due to the subendothelial deposits composed of a homogeneous fibrin fibrinogen-like substance and platelets without the formation of polymerized fibrin; (2) vascular wall thickening due to the intramural granular deposits of IGM and beta 1C. A patient with hemolytic-uremic syndrome 9 days after the onset of the disease, exhibited (1) the formation of fibrin-thrombi in the glomerular capillary lumens, and (2) the granular deposits of IgM and beta 1C along the glomerular capillary walls and in the mesangium. In contrast, the case in which the symptoms subsided five weeks after the onset exhibited neither fibrin-thrombus formation nor the deposits of IgM and beta 1C in the glomeruli. The vascular lesions of disseminated intravascular coagulation accompanied by pancreas carcinoma was located mainly in the capillaries, and were characterized by the formation of numerous fibrin-thrombi. Although the glomeruli contained numerous thrombi, there was neither endocapillary proliferation nor deposits of immunoglobulins and complement components.

摘要

血栓性血小板减少性紫癜的血管病变特征为心脏和大脑小动脉出现两种不同类型的变化

(1)由于由均质的纤维蛋白-纤维蛋白原样物质和血小板组成的内皮下沉积物导致多处不完全血管闭塞,未形成聚合纤维蛋白;(2)由于IgM和β1C的壁内颗粒状沉积物导致血管壁增厚。一名溶血性尿毒症综合征患者在发病9天后,表现为:(1)肾小球毛细血管腔内形成纤维蛋白血栓;(2)沿肾小球毛细血管壁和系膜有IgM和β1C的颗粒状沉积物。相比之下,发病五周后症状缓解的病例,肾小球内既未形成纤维蛋白血栓,也未出现IgM和β1C的沉积。伴有胰腺癌的弥散性血管内凝血的血管病变主要位于毛细血管,其特征是形成大量纤维蛋白血栓。尽管肾小球含有大量血栓,但既无毛细血管内增生,也无免疫球蛋白和补体成分的沉积。

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