2021年12月10日 · Although PNH cells are readily identified by flow cytometry due to their deficiency of GPI-anchored proteins, the assessment of the clinical significance of a PNH clone is more nuanced. The interpretation of results requires an understanding of PNH pathogenesis and its relationship to immune-mediated bone marrow failure.

2024年12月23日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare blood disorder which is caused by mutations in phosphatidylinositol glycan class A leading to hemolysis of red blood cells via complement inhibition. The first treatment for PNH, eculizumab, was FDA approved in 2007. Since then, many new treatment options for PNH have arisen.

The treatment that has recently become available is the complement blockade by the anti-C5 monoclonal antibody eculizumab. In this consensus, we are aiming to review the diagnosis and treatment of the paroxysmal nocturnal hemoglobinuria patients, as well as the early recognition of its systemic complications.

In this large single-centre study, we report high prevalence (25%) of, small (<10%) and very small (<1%), paroxysmal nocturnal hemoglobinuria (PNH) clones by high-sensitive cytometry among 3085 patients tested. Given PNH association with bone marrow failures, we analyzed 869 myelodysplastic sy …

阵发性睡眠性血红蛋白尿症（PNH）是由位于X染色体上的PIG-A基因突变导致的获得性造血干细胞克隆性疾病，其病理缺陷是糖基磷脂酰肌醇（GPI）合成异常而致由GPI锚连在血细胞膜上的一组锚连蛋白（CD55、CD59等）缺失，临床主要表现为血管内溶血、骨髓衰竭、高风险并发血栓等 [1, 2, 3]。 PNH经典的诊断方法已不能满足临床所需，被以流式细胞术为代表的现代检测手段而取代；治疗方面，糖皮质激素仍是治疗PNH控制溶血发作的首选，疗效确切，但对激素无效或依 …

Mutational spectrum and clonal hierarchy of patients with paroxysmal nocturnal hemoglobinuria (PNH). a Bar charts summarize the frequencies of mutations in the three groups: myelodysplastic syndromes (MDS; n = 835), hemolytic PNH (PNH; n = 83), and aplastic anemia (AA) and AA/PNH (AA+AA/PNH; n = 160).

Materials and methods: We evaluated the presence of CD55 negative and/or CD59 negative red blood cell (RBC) populations in newly diagnosed treatment naive-54 chronic lymphocytic leukemia (CLL) and 29 MM patients by flow cytometry.

2021年3月11日 · Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, clonal, complement-mediated hemolytic anemia with protean manifestations. PNH can present as a hemolytic anemia, a form of bone marrow failure, a thrombophilia, or any combination of the above.

Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon but frequently debilitating disease that, if untreated, may lead to death in up to 35% of patients within 5 years. Assessment of PNH clone size by flow cytometric analysis has increased in importance with the availability of therapeutic treatm …

2024年12月16日 · Introduction: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoietic stem cells, characterized by somatic mutations of the Phosphatidylinositol Glycan Class A Gene, resulting in increased hemolysis.