IMMUNOPHENOTYPIC ANALYSIS OF RETICULOCYTES IN PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA
IMMUNOPHENOTYPIC ANALYSIS OF RETICULOCYTES IN PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA
复制标题
DOI：
10.1182/blood.v86.4.1586.bloodjournal8641586
复制
发表时间：
1995-08-15
期刊：
影响因子：
20.3
通讯作者：
HALL, SE
中科院分区：
文献类型：
--
作者：
WARE, RE;ROSSE, WF;HALL, SE
血液系统疾病阵发性睡眠性血红蛋白尿症 (PNH) 是在骨髓干细胞内 Piga 基因发生获得性体细胞突变后发生的。这种突变细胞的后代无法合成糖基磷脂酰肌醇 (GPI) 锚，从而导致所有 GPI 连接蛋白的表面表达缺陷。 PNH 千变万化的临床表现可能是由于这些 GPI 连接表面蛋白的缺乏所致。为了解释中性粒细胞受影响的百分比显着高于循环红细胞的观察结果，并分析 PNH 中红细胞生成的增殖率，我们使用流式细胞术研究了 25 名患者。荧光染料噻唑橙用于检测网织红细胞，CD59单克隆抗体用于识别GPI缺陷细胞。与成熟的循环红细胞相反，异常网织红细胞的百分比与受影响的中性粒细胞的百分比相似。然而，绝大多数网织红细胞完全缺乏 GPI。即，即使在只有少量循环 III 型红细胞的患者中，也是 III 型细胞。此外，尽管在 25 名患者中的 10 名中发现了超过 5% 的 II 型成熟红细胞，但仅在 3 名患者中发现了超过 5% 的 II 型网织红细胞。结果表明，红系和中性粒细胞骨髓前体细胞在 PNH 中具有相当的增殖优势。该数据对于 PNH 中 II 型红细胞的起源也具有重要意义。 (C) 1995 年，美国血液学会。
The hematologic disorder paroxysmal nocturnal hemoglobinuria (PNH) occurs following an acquired somatic mutation in the Piga gene within a bone marrow stem cell. The progeny of this mutated cell cannot synthesize glycosylphosphatidylinositol (GPI) anchors, with a resultant deficiency in surface expression of all GPI-linked proteins. The protean clinical manifestations of PNH presumably result from the deficiency of these GPI-linked surface proteins. To explain the observation that neutrophils are affected at a significantly higher percentage than circulating erythrocytes and to analyze the proliferative rates of erythroid production in PNH, we studied 25 patients using flow cytometry. The fluorescent dye thiazole orange was used to detect reticulocytes, and CD59 monoclonal antibody was used to identify GPI-deficient cells. In contrast to the mature circulating erythrocytes, the percentage of abnormal reticulocytes was similar to the percentage of affected neutrophils. However, the vast majority of reticulocytes was completely GPI-deficient. ie, were type III cells, even in patients with only modest numbers of circulating type III erythrocytes. In addition, greater than 5% type II reticulocytes were identified in only 3 patients, although greater than 5% type II mature erythrocytes were identified in 10 of 25 patients. The results show that the erythroid and neutrophil bone marrow precursors have an equivalent proliferative advantage in PNH. The data also have important implications for the origin of type-II erythrocytes in PNH. (C) 1995 by The American society of Hematology.