阵发性睡眠性血红蛋白尿症（paroxysmal nocturnal hemoglobinuria, PNH）是一种由于1个或几个 造血干细胞 经 获得性 体细胞 PIG-A基因（phosphotidyl inositol glycan complementation group A）突变造成的非恶性的 克隆性 疾病，PIG-A突变造成 糖基磷脂酰肌醇 （glycosyl phosphatidyl inositol，GPI）合成异常，导致由GPI锚接在 细胞膜 上的一组 膜蛋白 丢失，包括CD16、CD55、CD59等，临床上主要表现为慢性 血管内溶血， 造血功能 衰竭和反复 血栓形成。

2023年7月31日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease that presents clinically with a variety of nonspecific symptoms. It causes complications mainly through intravascular hemolysis, thrombosis, and bone marrow failure.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, [1] life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system.

陣發性夜間血紅素尿症 （英語： paroxysmal nocturnal hemoglobinuria，縮寫為 PNH）是一種罕見、複雜且為後天造成的致命性 血液 疾病 [3]，被發現在 19世紀，大約每百萬人會有一到二人罹患此疾病 [4][5]，而確診後5年內的存活率只有約65% [6]。

2007年4月1日 · Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disease that results from nonmalignant clonal expansion of one or more hematopoietic stem cells that have undergone somatic mutation of the X-chromosome gene PIGA1, 2, 3 (Fig. 1).

Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic, multi-systemic, progressive and life-threatening disease characterized by intravascular hemolysis, thrombotic events, serious infections and bone marrow failure. 1, 2 Hemolysis in PNH is due to the action of the complement on abnormal red blood cells (RBCs).

2025年3月19日 · Paroxysmal nocturnal haemoglobinuria (PNH) is a haemolytic anaemia characterised by evidence of intravascular haemolysis such as haemoglobinuria and elevation of plasma lactate dehydrogenase. Diagnosis can be made by flow cytometry of granulocytes and red blood cells, looking for the lack of the ...

In routine analysis, RBCs can be identified by their light scatter properties; log/log displays of forward and side scatter are superior for identifying the RBC population because it is easier to exclude debris, especially platelet debris, on such plots, and significant aggregation, if present, can be more readily assessed.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare intravascular hemolytic anemia caused by an acquired mutation in the phosphatidylinositol-N-acetylglucosaminyltransferase-subunit-A (PIG-A) gene. This mutation leads to the deficiency of cellular anchors for complement inhibitor proteins cluster of differentiation (CD)55 and CD59, predisposing ...

2024年10月30日 · 阵发性睡眠性血红蛋白尿症（PNH）是一种罕见的血液系统疾病，可引发溶血、骨髓衰竭和血栓并发症等，严重者可危及生命。 长期以来，PNH面临着诊断困难的临床挑战，延迟诊断现状亟待改善。 最新发布的《阵发性睡眠性血红蛋白尿症诊断与治疗中国指南（2024年版）》提出了更为详细的诊断标准和流程1，进一步改善了患者预后。 值此契机，医脉通诚邀 四川大学华西医院何川教授 结合指南更新要点分享对PNH诊断与分型的独到见解。 由于PNH临床表 …