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阵发性睡眠性血红蛋白尿症(以下称PNH)是后天获得的以持续性血管内溶血为特征的干细胞疾病,至今原因未明。主要临床表现为严重的贫血和程度不同的白细胞及血小扳减少,有的病人可出现典型的阵发性的与睡眠相关的酱油色尿,有时在短时间内可使血红蛋白急剧下降。目前尚无特效的治疗方法,输血乃为延长病人寿命和减轻症状的重要治疗措施。但由于本病发病机理的某些特点,输血亦有相应的问题,若临床医师注意不够,输血不但不会改善病情,有时反而引起致命性的溶血发作。因此认真讨论和探讨本
Paroxysmal nocturnal hemoglobinuria (hereinafter referred to as PNH) is acquired acquired stem cell disease characterized by persistent intravascular hemolysis, so far the reason is unknown. The main clinical manifestations of severe anemia and varying degrees of leukopenia and thrombocytopenia decreased, and some patients may occur typical paroxysmal sleep-related soy sauce urine, and sometimes in a short period of time can make a sharp decline in hemoglobin. There is no effective treatment, blood transfusion is to prolong the life of patients and reduce the symptoms of important treatment. However, due to some characteristics of the pathogenesis of this disease, blood transfusion also has corresponding problems. If clinicians pay insufficient attention, blood transfusions will not only improve the condition but sometimes cause fatal hemolytic seizures. So seriously discuss and explore this