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男,26岁。因头晕、面黄、鲜酱油色尿、发热4d入院,2年前曾在某院诊断为“溶血-尿毒综合征”。查体:T37.8℃,柯兴氏面容,贫血貌,皮肤巩膜黄染,未见出血点及瘀斑,浅表淋巴结未及,咽稍红,两肺呼吸音清。HR100次/min,律齐,心尖部闻及Ⅲ级SM,腹软,肝脾未及,两下肢轻度浮肿。神经系统检查无阳性体征。血Hb 53g/L,RBC 1.21·10~(12)/L,WBC 12.7·10~9/L,PLT 98.0×10~9/L,Ret 0.066,分类正常,可见晚幼红细胞。骨髓检查示:增生性贫血骨髓象。血总胆红素106.5μmol/L,间接胆红素76.6μmol/L,直接胆红素正常。肾功能正常,尿蛋白(++),白细胞(++),尿隐血试验(卌),尿胆红素(+),尿罗氏试验(+),Coombs试验(-),Ham试验(+),血浆游离血红蛋白浓度420mg/L,蛇毒溶血度48%。诊断为阵发性睡眠性血
Male, 26 years old. Due to dizziness, face yellow, fresh soy sauce color urine, fever 4d admission, 2 years ago in a hospital diagnosed as “hemolysis - uremic syndrome.” Examination: T37.8 ℃, Cushing’s face, anemia appearance, skin scleral yellow dye, no bleeding spots and ecchymosis, superficial lymph nodes, throat slightly red, lung breath sounds clear. HR100 times / min, law Qi, apical Ministry smell and Ⅲ SM, abdominal soft, liver and spleen not yet, two lower limbs mild edema. Neurological examination no positive signs. Blood Hb 53g / L, RBC 1.21 · 10-12 / L, WBC 12.7 · 10-9 / L, PLT 98.0 × 10-9 / L, Ret 0.066, normal classification, visible late young erythrocytes. Bone marrow examination showed: hyperplastic anemia bone marrow. Blood total bilirubin 106.5μmol / L, indirect bilirubin 76.6μmol / L, direct bilirubin normal. Urinary protein (++), white blood cells (++), urine occult blood test (卌), urinary bilirubin (+), urine Roche test (+), Coombs test (-), Ham test , Plasma free hemoglobin concentration 420mg / L, snake venom hemolysis 48%. Diagnosis of paroxysmal nocturnal blood