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阵发性夜间血红蛋白尿(PNH)中的胎儿血红蛋白:含HbF红细胞(F细胞)源自PNH克隆以及正常造血干细胞系的证据。

Fetal hemoglobin in paroxysmal nocturnal hemoglobinuria (PNH): evidence for derivation of HbF-containing erythrocytes (F cells) from the PNH clone as well as from normal hemopoietic stem cell lines.

作者信息

Papayannopoulou T, Rosse W, Stamatoyannopoulos G

出版信息

Blood. 1978 Oct;52(4):740-9.

PMID:356915
Abstract

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.

摘要

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。

相似文献

1
Fetal hemoglobin in paroxysmal nocturnal hemoglobinuria (PNH): evidence for derivation of HbF-containing erythrocytes (F cells) from the PNH clone as well as from normal hemopoietic stem cell lines.阵发性夜间血红蛋白尿(PNH)中的胎儿血红蛋白:含HbF红细胞(F细胞)源自PNH克隆以及正常造血干细胞系的证据。
Blood. 1978 Oct;52(4):740-9.
2
Expression of blood group i antigen and fetal hemoglobin in paroxysmal nocturnal hemoglobinuria.阵发性夜间血红蛋白尿症中血型i抗原和胎儿血红蛋白的表达
Transfusion. 1997 Mar;37(3):291-7. doi: 10.1046/j.1537-2995.1997.37397240211.x.
3
Separation of the acetylcholinesterase-deficient red cells in paroxysmal nocturnal hemoglobinuria.阵发性夜间血红蛋白尿中乙酰胆碱酯酶缺乏红细胞的分离
Blood. 1986 Apr;67(4):893-7.
4
Specific defect in N-acetylglucosamine incorporation in the biosynthesis of the glycosylphosphatidylinositol anchor in cloned cell lines from patients with paroxysmal nocturnal hemoglobinuria.阵发性夜间血红蛋白尿症患者克隆细胞系中糖基磷脂酰肌醇锚生物合成过程中N-乙酰葡糖胺掺入的特异性缺陷。
Proc Natl Acad Sci U S A. 1993 Jun 1;90(11):5272-6. doi: 10.1073/pnas.90.11.5272.
5
Decay-accelerating factor is present on paroxysmal nocturnal hemoglobinuria erythroid progenitors and lost during erythropoiesis in vitro.衰变加速因子存在于阵发性夜间血红蛋白尿症红细胞祖细胞上,并在体外红细胞生成过程中丢失。
J Exp Med. 1985 Oct 1;162(4):1182-92. doi: 10.1084/jem.162.4.1182.
6
Characterization of the complement sensitivity of paroxysmal nocturnal hemoglobinuria erythrocytes.阵发性夜间血红蛋白尿症红细胞补体敏感性的特征分析
J Clin Invest. 1985 Jun;75(6):2074-84. doi: 10.1172/JCI111927.
7
The PIG-A mutation and absence of glycosylphosphatidylinositol-linked proteins do not confer resistance to apoptosis in paroxysmal nocturnal hemoglobinuria.阵发性睡眠性血红蛋白尿症中的PIG-A突变及糖基磷脂酰肌醇连接蛋白缺失并不赋予细胞对凋亡的抗性。
Blood. 1998 Oct 1;92(7):2541-50.
8
Altered expression of gangliosides in erythrocytes of paroxysmal nocturnal hemoglobinuria.阵发性夜间血红蛋白尿患者红细胞中神经节苷脂表达的改变。
J Clin Invest. 1990 May;85(5):1456-61. doi: 10.1172/JCI114591.
9
Circulating primitive stem cells in paroxysmal nocturnal hemoglobinuria (PNH) are predominantly normal in phenotype but granulocyte colony-stimulating factor treatment mobilizes mainly PNH stem cells.阵发性睡眠性血红蛋白尿(PNH)中循环的原始干细胞在表型上主要是正常的,但粒细胞集落刺激因子治疗主要动员PNH干细胞。
Blood. 1998 Jun 15;91(12):4504-8.
10
Confirmation of a structural abnormity in the stroma of erythrocytes from paroxysmal nocturnal hemoglobinuria (PNH) after hemolysis in distilled water.阵发性睡眠性血红蛋白尿症(PNH)患者红细胞在蒸馏水中溶血后,其基质结构异常得到证实。
Blood. 1956 Aug;11(8):753-6.

引用本文的文献

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Heterogeneity of fetal hemoglobin production in adult red blood cells.成人红细胞中胎儿血红蛋白生成的异质性。
Curr Opin Hematol. 2021 May 1;28(3):164-170. doi: 10.1097/MOH.0000000000000640.
2
Understanding heterogeneity of fetal hemoglobin induction through comparative analysis of F and A erythroblasts.通过比较 F 和 A 红细胞来理解胎儿血红蛋白诱导的异质性。
Blood. 2020 May 28;135(22):1957-1968. doi: 10.1182/blood.2020005058.
3
Stochastic expression of fetal hemoglobin in adult erythroid cells.成体红细胞中胎儿血红蛋白的随机表达。
Proc Natl Acad Sci U S A. 1981 Nov;78(11):7005-9. doi: 10.1073/pnas.78.11.7005.
4
The modulation of Hb F synthesis in adult erythroid progenitor (burst-forming unit) cultures reflects changes in gamma-globin gene transcription and chromatin structure.成人红系祖细胞(爆式集落形成单位)培养物中Hb F合成的调节反映了γ-珠蛋白基因转录和染色质结构的变化。
Proc Natl Acad Sci U S A. 1986 Sep;83(18):6887-91. doi: 10.1073/pnas.83.18.6887.