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脾脏及脾切除术在自身免疫性溶血性疾病中的作用

The role of the spleen and splenectomy in autoimmune hemolytic disease.

作者信息

Bowdler A J

出版信息

Semin Hematol. 1976 Oct;13(4):335-48.

PMID:1006334
Abstract

The relationship of the spleen to the red cell in AHDW is determined by the characteristics of the surface protein coating of the red cell, rather than by a pathologic change in the reactivity of the spleen. The behavior of the spleen is essentially a passive response to red cells with abnormal properties. The critical properties are incompletely defined, but probably include cell shape, and perhaps more importantly, the intrinsic properties of the cell membrane. The effects produced by the spleen are mediated partly by the filtration mechanism of the splenic sinus walls, and partly by the more specific effects of cell-to-cell adhesion between the pulp macrophages and the red cells. The outcome is more complex than former concepts of red cell destruction encompassed: delay in cell passage produces cell pooling in the unfavorable environment of the splenic pulp, and there is microfragmentation, cell sphering, and partial phagocytosis, in addition to cell destruction with erythrophagocytosis in situ. Prediction of the degree of dependence of hemolysis on the spleen prior to splenectomy remains an area of uncertainty. Commonly, the decision for splenectomy is determined circumstantially, but current concepts would anticipate that the most relevant data would relate to the dimensions of splenic pooling, and the quantity and class of immunoglobulin present on the cells.

摘要

在非自身免疫性溶血性贫血(AHDW)中,脾脏与红细胞的关系取决于红细胞表面蛋白包被的特性,而非脾脏反应性的病理变化。脾脏的行为本质上是对具有异常特性的红细胞的被动反应。关键特性尚未完全明确,但可能包括细胞形状,或许更重要的是细胞膜的内在特性。脾脏产生的效应部分是通过脾窦壁的过滤机制介导的,部分是通过脾髓巨噬细胞与红细胞之间细胞间黏附的更特异性效应介导的。其结果比以往关于红细胞破坏的概念所涵盖的更为复杂:细胞通过延迟会导致细胞在脾髓的不利环境中聚集,除了原位红细胞吞噬导致的细胞破坏外,还会出现微碎片形成、细胞球形化和部分吞噬作用。在脾切除术前预测溶血对脾脏的依赖程度仍然是一个不确定的领域。通常,脾切除术的决定是根据具体情况做出的,但目前的概念预计,最相关的数据将与脾脏聚集的程度以及细胞上存在的免疫球蛋白的数量和类别有关。

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The role of the spleen and splenectomy in autoimmune hemolytic disease.脾脏及脾切除术在自身免疫性溶血性疾病中的作用
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引用本文的文献

1
Treatment Options for Primary Autoimmune Hemolytic Anemia: A Short Comprehensive Review.原发性自身免疫性溶血性贫血的治疗选择:简短综述
Transfus Med Hemother. 2015 Sep;42(5):294-301. doi: 10.1159/000438731. Epub 2015 Aug 10.
2
Diagnosis and treatment of autoimmune haemolytic anaemias in adults: a clinical review.成人自身免疫性溶血性贫血的诊断与治疗:临床综述
Wien Klin Wochenschr. 2008;120(5-6):136-51. doi: 10.1007/s00508-008-0945-1.
3
Splenectomy for hematologic disease. The UCLA experience with 306 patients.血液系统疾病的脾切除术。加州大学洛杉矶分校对306例患者的经验。
Ann Surg. 1984 Jul;200(1):40-5. doi: 10.1097/00000658-198407000-00006.
4
Elective splenectomy in haematological disorders.血液系统疾病中的择期脾切除术。
Ann R Coll Surg Engl. 1988 Jan;70(1):29-33.
5
Impaired regulation of erythrocyte autoantibody production after splenectomy.脾切除术后红细胞自身抗体产生的调节受损。
Br J Exp Pathol. 1979 Oct;60(5):466-70.