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目的总结以产气单胞菌溶素前体变异体(FLAER)技术诊断的阵发性睡眠性血红蛋白尿症(PNH)患者的临床特点,提高对PNH的认识。方法回顾性分析2011年9月至2015年3月在南通大学第三附属医院血液科和江苏省人民医院血液科经FLAER诊断的98例以流式细胞术FLAER技术诊断的PNH患者资料,对临床表现、实验室检查结果、并发症进行分析归纳。结果通过FLAER技术诊断经典型PNH 43例,PNH合并骨髓疾病45例,亚临床型PNH 10例。70例有疲乏表现,41例以血红蛋白尿为主要表现,6例合并血栓形成(多为非常见部位),17例发生肾功能损害,19例合并肝功能损害,肺动脉高压仅2例。经典型PNH更易发生溶血,PNH合并骨髓疾病者造血功能衰竭特征较明显。增高的乳酸脱氢酶(LDH)值与PNH粒细胞克隆数间存在线性关系,且PNH粒细胞克隆数较高者更易发生血栓。结论 PNH患者以溶血及骨髓衰竭为主要表现,易见肝肾损伤及少见部位血栓,肺动脉高压少见。
OBJECTIVE: To summarize the clinical features of patients with paroxysmal nocturnal hemoglobinuria (PNH) diagnosed with the aerosolv precursor variant (FLAER) technique and to improve their understanding of PNH. Methods A retrospective analysis of 98 patients with PNH diagnosed by FLAER hematology department of the Third Affiliated Hospital of Nantong University and FLAER of Jiangsu Provincial People’s Hospital from September 2011 to March 2015 was performed on clinical data Performance, laboratory test results, complications were analyzed and summarized. Results FLAER technique was used to diagnose classic PNH in 43 cases, PNH combined with bone marrow disease in 45 cases and subclinical PNH in 10 cases. 70 patients had fatigue, 41 patients had hemoglobinuria as the main manifestation, 6 patients had thrombosis (mostly uncommon), 17 patients had renal dysfunction, 19 patients had hepatic dysfunction and only 2 patients had pulmonary hypertension. Classic PNH more susceptible to hemolysis, PNH complicated with bone marrow disease, hematopoietic failure characteristics more obvious. There was a linear relationship between increased lactate dehydrogenase (LDH) value and PNH neutrophil clones, and higher PNH neutrophil clots were more likely to develop thrombus. Conclusions Hemolytic and bone marrow failure are the main manifestations in patients with PNH. It is easy to see liver and kidney injury, rare thrombosis and pulmonary hypertension.