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 (PNH) is a nonmalignant clonal hematopoietic disorder characterized by the lack of glycosylphosphatidylinositol-anchored proteins (GPI-APs) as a consequence of somatic mutations in the phosphatidylinositol glycan anchor biosynthesis class A (PIGA) gene. Clini...
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 ...
Orphan Drugs: Research and Reviews Dovepress open access to scientific and medical research Open Access Full Text Article Paroxysmal nocturnal hemoglobinuria: new concepts in pathophysiology and treatment Review This article was published in the following Dove Press journal: Orphan Drugs: Research and ...
Paroxysmal nocturnal haemoglobinuria is an ultra-rare disease and potentially life-threatening disease. Learn more about the causes, symptoms, and unmet needs.
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 ...
Miller, "Paroxysmal nocturnal hemoglobinuria (PNH) and pregnancy in the era of eculizumab," Leukemia Research, vol. Paroxysmal Nocturnal Hemoglobinuria in Pregnancy: A Dilemma in Treatment and Thromboprophylaxis * The report reviews key players involved in the therapeutics development for Paroxysmal Nocturn...
Nocturnal Hot Flashes and Alzheimer's Risk Highlights on Advances in the Treatment of Paroxysmal Nocturnal Hemoglobinuria From ASH 2024 Conference MDAngle: ASH 2024 Paroxysmal Nocturnal Hemoglobinuria ASH 2024: New Data in Paroxysmal Nocturnal Hemoglobinuria Tools Drug Interaction Checker Pill Identifie...
Treatment of paroxysmal nocturnal hemoglobinuria in the era of eculizumab Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening and debilitating clonal blood disorder caused by an acquired mutation in the phosphat... A R?Th,U Dührsen - 《European Journal of Haematology》 被引量: ...
REVIEW Paroxysmal nocturnal hemoglobinuria: pathophysiology, natural history and treatment options in the era of biological agents Antonio M Risitano Bruno Rotoli Hematology, Department of Biochemistry and Medical Biotechnologies, Federico II University of Naples, Italy Abstract: Paroxysmal nocturnal ...