Papayannopoulou T, Rosse W, Stamatoyannopoulos G
Blood. 1978 Oct;52(4):740-9.
The cellular distribution of HbF was studied in nine patients with paroxysmal nocturnal hemoglobinuria (PNH) by measuring the level of HbF and determining the number of HbF-containing red cells (F cells) in whole blood and in the population of normal cells obtained after immune lysis of the abnormal erythrocytes. The amounts of HbF and the F cell frequencies found in the normal red cells were strikingly similar to the values seen in whole blood. The observed frequencies of F cells in normal cells best fitted those expected under the assumption that the F cells arise equally from normal hemopoietic stem cells and from the stem cells with the PNH defect. Since PNH appears to be a clonal hemopoietic stem cell disorder, this evidence argues against a derivation of F cells from distinct pluripotent stem cell lines.
通过测量血红蛋白F(HbF)水平并确定全血及异常红细胞免疫裂解后获得的正常细胞群体中含HbF红细胞(F细胞)的数量,研究了9例阵发性夜间血红蛋白尿(PNH)患者的HbF细胞分布情况。在正常红细胞中发现的HbF量和F细胞频率与全血中的值惊人地相似。在正常细胞中观察到的F细胞频率最符合在以下假设下预期的频率,即F细胞同样来源于正常造血干细胞和具有PNH缺陷的干细胞。由于PNH似乎是一种克隆性造血干细胞疾病,这一证据反对F细胞来源于不同多能干细胞系的观点。