PNH是一种由后天基因突变造成并导致红细胞破裂(医学上称“溶血”)的血液疾病。一般来说,正常红细胞膜上有几种保护性蛋白,可使红细胞免受机体的补体系统(先天免疫系统的一部分)误伤。然而,PNH患者造血干细胞的PIG-A基因发生了突变,突变后血细胞膜表面的锚连蛋白缺失,血细胞失去这些蛋白的保护,对补体高度敏感,进而...
Paroxysmal nocturnal hemoglobinuria occurs due to the development of a genetic mutation in hematopoietic stem cells. This mutation of the X-linked gene phosphatidylinositol glycan class A (PIGA), produces a deficiency in the glycosylphosphatidylinositol (GPI) protein, which is responsible for anchoring ...
Rosse WF. Paroxysmal nocturnal hemoglobinuria: the biochemical defects and the clinical syndrome. Blood Rev 1989; 3: 192–200.Rosse, W.F. ( 1989 ) Paroxysmal nocturnal hemoglobinuria. The biochemical defects and the clinical syndrome . Blood Reviews , 3 , 192 200 ....
nocturnal paroxysmal hemoglobinuria夜间阵发性血红蛋白尿症 paroxysmal nocturnal hemoglobinuria阵发性睡眠性血红蛋白尿,阵发性夜间血红蛋白尿 nocturnal hemoglobinuria夜间血红蛋白尿 paroxysmal hemoglobinuria阵发性血红蛋白尿症 paroxysmal cold hemoglobinuria阵发性寒冷性血红蛋白尿症 ...
nocturnal paroxysmal hemoglobinuria夜间阵发性血红蛋白尿症 paroxysmal nocturnal hemoglobinuria阵发性睡眠性血红蛋白尿,阵发性夜间血红蛋白尿 nocturnal hemoglobinuria夜间血红蛋白尿 paroxysmal hemoglobinuria阵发性血红蛋白尿症 paroxysmal cold hemoglobinuria阵发性寒冷性血红蛋白尿症 ...
阵发性睡眠性血红蛋白尿(paroxysmal nocturnal hemoglobinuria,PNH)系后天获得性的红细胞膜缺陷引起的对补体激活异常敏感的慢性血管内溶血,常睡眠时加重,可伴间歇性血红蛋白尿和全血细胞减少症或反复血栓形成。 病因和发病机制 PNH 是由于红细胞膜上缺乏抑制补体激活及膜反应性溶解的蛋白质所致。该类膜蛋白夹有十余种,...
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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired stem cell disorder (incidence of 2–6 per million) characterized by hemolytic anemia, marrow failure and thrombosis. It is due to a mutation in the phosphatidylinositol glycan class A (PIG-A) gene, which results in a deficiency of gl...
Research and Reviews 1 September 2015 Number of times this article has been viewed Ilene Ceil Weitz Jane Anne Nohl Division of Hematology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA Abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological dis...
Paroxysmal nocturnal haemoglobinuria is an ultra-rare disease and potentially life-threatening disease. Learn more about the causes, symptoms, and unmet needs.