2012年5月11日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder characterized by hemolytic anemia. It arises due to a somatic mutation in the PIGA gene, causing deficiency of glycosylphosphatidylinositol-anchored proteins (GPI-APs) like CD55 and CD59 on the surface of blood cells.

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 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder characterized by hemolytic anemia. It arises due to a somatic mutation in the PIGA gene, causing deficiency of glycosylphosphatidylinositol-anchored proteins like CD55 and CD59 on the surface of blood cells.

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 · The paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired bone marrow failure disorder, which leads to premature death and impaired red blood cells (RBCs). PNH is characterized by hemolytic anemia, thrombosis, and peripheral blood cytopenia.

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. In fact, nocturnal hematuria only occurs in about 25-50% of pts.

2014年7月13日 · Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare and serious blood disorder characterized by the destruction of red blood cells, leading to various complications.

2017年2月3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 …

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 …

2020年9月26日 · Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare, acquired hemolytic anemia caused by a loss of proteins that regulate the complement pathway, making red blood cells sensitive to complement-mediated lysis. It is characterized by intravascular hemolysis that is worse at night due to relative acidosis during sleep.