阵发性睡眠性血红蛋白尿(Paroxysmal mocturnal hemoglobinuria,PNH)属后天性红细胞膜缺陷疾病,表现为对补体溶血作用异常敏感,临床上以血管内溶血为特点,这种细胞缺陷也存在于血小板和白细胞,故PNH患者除贫血外还常有白细胞和血小板减少。该病在我国北方较常见。症状多不典型,特殊诊断方法又少,误诊者并非鲜见。我院收治的25例PNH中误诊者15例,误诊率达60%,本文就此进行分析讨论。一、误诊病例及其特点首发症状:发烧(2例)、黄疸(4例)、乏力(11例)、酱油尿(5例)、粘膜出血及/或皮肤紫癜(5例)、心慌(2例)、头晕(3例)、腰背疼(1例)。误诊疾病类型:肝炎(4例)、再障(6例)、肾炎(2例)、上消化道出血(1例),白细胞减 Paroxysmal mocturnal hemoglobinuria (PNH) is a congenital erythrocyte membrane disease, which is characterized by abnormal sensitivity to complement hemolysis and clinically intravascular hemolysis. This cell defect also exists in platelets and leukocytes , So PNH patients in addition to anemia often white blood cells and thrombocytopenia. The disease is more common in northern China. More atypical symptoms, special diagnostic methods and fewer misdiagnosis is not uncommon. In our hospital, 25 cases of PNH misdiagnosis in 15 cases, the misdiagnosis rate of 60%, this article on the analysis and discussion. First, the misdiagnosed cases and their characteristics The first symptom: fever (2 cases), jaundice (4 cases), fatigue (11 cases), soy sauce urine (5 cases), mucosal bleeding and / or purpura (5 cases), palpitation Cases), dizziness (3 cases), low back pain (1 case). Misdiagnosis of disease types: hepatitis (4 cases), aplastic anemia (6 cases), nephritis (2 cases), upper gastrointestinal bleeding (1 case), leukopenia