Since publication in 2010 of the International Clinical Cytometry Society (ICCS) Consensus Guidelines for detection of Paroxysmal nocturnal hemoglobinuria (PNH) by flow cytometery, a …

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by a somatic mutation in the PIGA gene, leading to a deficiency of proteins …

2024年11月1日 · 阵发性睡眠性血红蛋白尿症（PNH）是一种后天获得性造血干细胞克隆性疾病。 由于造血干细胞磷脂酰肌醇聚糖A（PIGA）基因突变，影响锚链蛋白糖基磷脂酰肌醇（GPI） …

ICCS Consensus 5 and Practical Guidelines 6 for detecting PNH phenotypes on RBC and WBC lineages have been published. RBC methodology consists of identifying RBC by the …

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 …

To detect GPI-deficient PNH RBC phenotypes, antibodies to CD59 provide the best means to delineate and enumerate PNH type III, PNH type II and normal (type I) RBCs 11, 19.

Paroxysmal nocturnal hemoglobinuria (PNH) arises as a consequence of the non-malignant clonal expansion of one or more hematopoietic stem cells with an acquired somatic mutation of the …

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by the expansion of one or more clones of stem cells producing progeny of mature blood cells deficient in the plasma …

2020年11月5日 · PIGA mutations lead to impaired biosynthesis of glycosylphosphoinositol (GPI)-anchor. Resultant lack of GPI-anchored proteins (GPI-AP) leads to a clonal expansion of PNH …

Flow cytometric analysis of GPI-anchored proteins (GPI-AP) is the gold standard for diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). Due to therapy options and the relevance of GPI …