Navenot J M, Muller J Y, Blanchard D
Etablissement de Transfusion Sanguine Loire Atlantique-Vendée, Nantes, France.
Transfusion. 1997 Mar;37(3):291-7. doi: 10.1046/j.1537-2995.1997.37397240211.x.
The mechanism by which the paroxysmal nocturnal hemoglobinuria (PNH) clone progressively takes over normal hematopoietic cells remains unknown. The respective in vivo differentiation of normal and PNH erythroid progenitors was investigated through the expression of two fetal erythroid markers (i antigen and fetal hemoglobin [HbF]) whose expression in adult red cells is associated with altered erythropoiesis.
Murine monoclonal antibodies directed against HbF and i and CD59 antigens were used to phenotype red cells of 10 PNH patients. A multiparametric flow cytometry assay of red cells and reticulocytes was designed to assess a possible association of i and HbF with PNH or normal red cells.
Most patients exhibited greater expression of i and HbF than did normal controls. In each case, the percentages of i-positive or HbF-positive cells within CD59-deficient and CD59-positive red cells were very close, clearly showing a lack of preferential association of these markers with normal or PNH cells.
In PNH patients, normal and PNH erythroid progenitors have the same ability to promote HbF and i antigen expression, which suggests that normal and PNH erythroid progenitors (burst-forming units-erythroid, colony-forming units-erythroid, erythroblasts) behave similarly in response to bone marrow stress.
阵发性睡眠性血红蛋白尿（PNH）克隆逐渐取代正常造血细胞的机制尚不清楚。通过两种胎儿红系标志物（i抗原和胎儿血红蛋白[HbF]）的表达来研究正常和PNH红系祖细胞在体内的各自分化情况，这两种标志物在成人红细胞中的表达与红细胞生成改变有关。
使用针对HbF、i和CD59抗原的鼠单克隆抗体对10例PNH患者的红细胞进行表型分析。设计了一种红细胞和网织红细胞的多参数流式细胞术检测方法，以评估i和HbF与PNH或正常红细胞之间可能存在的关联。
大多数患者的i和HbF表达高于正常对照组。在每种情况下，CD59缺陷型和CD59阳性红细胞中i阳性或HbF阳性细胞的百分比非常接近，清楚地表明这些标志物与正常或PNH细胞之间缺乏优先关联。
在PNH患者中，正常和PNH红系祖细胞促进HbF和i抗原表达的能力相同，这表明正常和PNH红系祖细胞（红系爆式集落形成单位、红系集落形成单位、成红细胞）在应对骨髓应激时表现相似。
