2012年5月11日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired blood disorder characterized by complement-induced hemolytic anemia, red urine, and thrombosis. It results from a genetic mutation causing blood cells to be missing a …

2018年4月1日 · PNH特点：PNH为慢性血管内溶血病，可有血红蛋白尿，可全血细胞减少，网织红细 胞增多，骨髓增生活跃，以红系增生为主，血管内溶血的证据， CD55/CD59减缺细胞比例大于10%。

2022年6月1日 · This document provides information on paroxysmal nocturnal hemoglobinuria (PNH). It describes PNH as an acquired hemolytic anemia caused by a somatic mutation in hematopoietic stem cells, rendering red blood cells susceptible to complement-mediated lysis. This results in intravascular hemolysis, thrombosis, and cytopenias.

2017年5月25日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder characterized by hemolytic anemia, bone marrow failure, and thrombosis. It results from a somatic mutation in the PIG-A gene, leading to a deficiency in glycosylphosphatidylinositol-anchored proteins on the surface of blood cells.

2014年8月22日 · Section 5 HA Paroxysmal Nocturnal Hemoglobinuria (PNH) Definition Etiology and pathogenesis Characteristics of PNH （clinical and lab） Diagnostic process Definition PNH is a clonal disorder of hematopoietic stem cells caused by a somatic mutation of the pig-A gene on the short arm of the X chromosome .

PNH is named for its most dramatic manifestation—intermmitent nocturnal hemolysis and morning hemoglobinuria, although this is a bit of a misnomer since it is not always paroxysmal (intermittent) and hemoglobinuria may occur at any time throughout the day.

PNH: Cerebral Vein Thrombosis, visceral thrombosis (liver), skin. Treated with chronic anticoagulation, but generally not prophylactically. Anita Hill, Richard J. Kelly, Peter Hillmen; …

2017年5月7日 · 造血干细胞移植治疗PNH 异基因骨髓移植是唯一能治愈本病方法 指征：PNH合併骨髓增生低下，且反复发生严重血管栓塞并发症者。 国外报导60例异基因BMT，75%获得造血功能恢复并长期生存。 自身造血干细胞移植 非清髓造血干细胞移植及MUD BMT Eculizumab 人源性C5单抗，可抑制C5转化酶，阻止MAC形成 疗效：11例依赖输血PNH, Eculizumab 600mg i.v. qw×4，一周后900mg i.v. qow 至w12，溶血控制，可以基本不输血，生活质量改善 ( NEJM …

2018年1月8日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare blood disorder characterized by the breakdown of red blood cells in the bloodstream, causing dark urine. It results from a genetic mutation in stem cells, making red blood cells vulnerable to destruction by the immune system.

2014年11月18日 · In PNH, something allows the abnormal cells to become the dominant cells and become the major population in the marrow (anywhere from 1 to over 90% - referred to as the clone size).