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患儿女性,9岁。因上腹部疼痛不适2月余伴头晕乏力1周于1992年9月19日第1次住本院。体检:T37.8℃,重度贫血貌,巩膜无黄染,双侧颌下、腋窝可及浅表淋巴结,无红肿压痛,心肺听诊正常,肝脏肋下1cm,脾肋下1.5cm,皮肤无皮疹,四肢关节正常。入院时血白细胞数6.5×10~9/L,血红蛋白47g/L,血小板计数148×10~9/L,网织红细胞17.4%,入院后患儿一直感胸闷,头晕,并出现高热,达40℃,伴尿色深黄,肝脾进行性增大,血红蛋白急剧下降,网织红细胞明显增高,尿胆原±~++,肝功能:总胆红素37.6μmol/L,直接胆红素1.7μmol/L,Comb’s试验直接反应阳性,间接反应阴性,类风湿因子(+),抗DNA结合率2%,抗核抗体(+),骨髓报告系增生性贫血骨髓象。诊断为自身免疫性溶血性贫血。予激素及输入洗涤红细胞等治疗。其间患儿溶血曾反复2次。3个月后,患儿溶血基本控制,血红蛋白上升至146g/L,网织红细胞3%。予强的松口服维持治疗出院。5个月后,患儿因发热伴头晕乏力2天第2次住入本院。体检:柯兴氏貌,T39℃,
Children with children, 9 years old. Due to upper abdominal pain discomfort in February with dizziness and fatigue for 1 week on September 19, 1992 first hospital. Physical examination: T37.8 ℃, severe anemia, sclera without yellow dye, bilateral submandibular, axillary and superficial lymph nodes, no redness tenderness, normal auscultation of the heart and lung, the ribs 1cm under the ribs, 1.5cm under the splenic ribs, no skin rash , Limbs and joints normal. On admission, the number of white blood cells 6.5 × 10 ~ 9 / L, hemoglobin 47g / L, platelet count 148 × 10 ~ 9 / L, reticulocytes 17.4%, admitted to the hospital has been chest tightness, dizziness and fever, up to 40 ℃, accompanied by dark yellow urine, liver and spleen progressively increased, a sharp decline in hemoglobin, reticulocyte was significantly increased, urobilinogen ± ~ ++, liver function: total bilirubin 37.6μmol / L, direct bilirubin 1.7 μmol / L, Comb’s test was positive for direct reaction, negative for indirect reaction, rheumatoid factor (+), anti-DNA binding rate of 2%, antinuclear antibody (+), myeloid bone marrow report of hyperplastic anemia. Diagnosis of autoimmune hemolytic anemia. Hormone and input washing red blood cells and other treatment. During which hemolysis was repeated 2 times. Three months later, hemolysis in children with basic control, hemoglobin rose to 146g / L, reticulocyte 3%. To prednisone oral maintenance treatment and discharge. Five months later, the patient was admitted to our hospital for the second time due to fever with dizziness and weakness for 2 days. Physical examination: Cushing’s appearance, T39 ℃,