Temporal differences in membrane loss lead to distinct reticulocyte features in hereditary spherocytosis and in immune hemolytic anemia
Temporal differences in membrane loss lead to distinct reticulocyte features in hereditary spherocytosis and in immune hemolytic anemia
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DOI：
10.1182/blood.v98.10.2894
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发表时间：
2001-11-15
期刊：
影响因子：
20.3
通讯作者：
Cynober, T
中科院分区：
文献类型：
--
作者：
Da Costa, L;Mohandas, N;Cynober, T
具有减少的膜表面积的球形红细胞是遗传性球形红细胞增多症（HS）和某些形式的自身免疫性溶血性贫血（AIHA）的特征。一般认为球形红细胞的膜损失发生在其停留在循环中。HS中膜损失的结构基础是膜蛋白的不适当组装，而在AIHA中，它是由于巨噬细胞对循环红细胞的部分吞噬作用。形成了一种假设，即这些不同的机制应导致这2种球形红细胞综合征在红细胞生成期间和在循环中逗留期间表面积损失的时间差异。有人提出，细胞表面损失可能开始在网织红细胞阶段在HS，而表面积损失在ANA只涉及循环成熟红细胞。通过记录HS和AIHA中网织红细胞细胞特征的差异，确立了这一假设的有效性。使用一种新的技术来定量细胞表面积，记录了与正常细胞相比，HIS中网织红细胞和成熟红细胞的膜表面积减少。相反，在AIHA中，只有成熟红细胞而不是网织红细胞表现出膜表面积减少。这些数据表明，HS中的表面积损失，而不是在ANA中，已经存在于循环网织红细胞阶段。这些发现意味着细胞表面积的损失是HS红细胞发生过程中的早期事件，并挑战了现有的概念，即HS中的表面积损失主要发生在成熟红细胞在循环中的逗留期间。(C)2001年，美国血液学会。
Spherocytic red cells with reduced membrane surface area are a feature of hereditary spherocytosis (HS) and some forms of autoimmune hemolytic anemia (AIHA). It is generally assumed that membrane loss in spherocytic red cells occurs during their sojourn in circulation. The structural basis for membrane loss in HS is improper assembly of membrane proteins, whereas In AIHA it is due to partial phagocytosis of circulating red cells by macrophages. A hypothesis was formed that these different mechanisms should lead to temporal differences in surface area loss during red cell genesis and during sojourn in circulation in these 2 spherocytic syndromes. It was proposed that cell surface loss could begin at the reticulocyte stage In HS, whereas surface area loss in ANA involves only circulating mature red cells. The validity of this hypothesis was established by documenting differences in cellular features of reticulocytes in HS and AIHA. Using a novel technique to quantitate cell surface area, the decreased membrane surface area of both reticulocytes and mature red cells in HIS compared with normal cells was documented. In contrast, in AIHA only mature red cells but not reticulocytes exhibited decreased membrane surface area. These data imply that surface area loss in HS, but not In ANA, is already present at the circulating reticulocyte stage. These findings imply that loss of cell surface area is an early event during genesis of HS red cells and challenge the existing concepts that surface area loss in HS occurs predominantly during the sojourn of mature red cells in circulation. (C) 2001 by The American Society of Hematology.