Okada S, Miyoshi Y, Takizawa Y, Hagiwara S, Mori H, Niikura H, Terada H, Watanabe H, Mitsuya T, Sagawa F
Rinsho Ketsueki. 1989 Oct;30(10):1892-7.
We report a rare case of fulminant autoimmune hemolytic anemia (AIHA) with multiple organ failure (MOF). A 40-year-old man was emergently admitted to our hospital because of conscious disturbance and jaundice. The peripheral blood revealed RBC 68 x 10(4)/microliter, Hb 3.5 g/dl, Ht 8.9%, Ret 30% (20,400/microliter), WBC 20,300/microliter, Plts 16.9 x 10(4)/microliter, indirect bilirubin 9.4 mg/dl. Both direct and indirect Coombs test were positive and the IgG autoantibody was identified. Bone marrow aspiration revealed hypercellularity with increased megakaryocytes and erythroid hyperplasia. The patient was diagnosed as having idiopathic warm type of AIHA and the therapy was started with prednisolone 80 mg/day from the first day of admission but hemolysis with reticulocytopenia was so rapidly progressive that he was in acutely life-threatening state and MOF (acute renal failure, adult respiratory distress syndrome, congestive heart failure, liver dysfunction, rhabdomyolysis) appeared on the third hospital day. Plasma exchange therapy and hemodialysis were started and high dose methylprednisolone was given soon after rapid administration of sufficient blood transfusion. Dramatic improvement of hemolysis was noted and MOF was controlled after starting these therapies, but he died of exacerbation of MOF probably due to sepsis 40 days later.
我们报告一例罕见的伴有多器官功能衰竭(MOF)的暴发性自身免疫性溶血性贫血(AIHA)。一名40岁男性因意识障碍和黄疸紧急入院。外周血检查显示红细胞计数68×10⁴/微升,血红蛋白3.5克/分升,血细胞比容8.9%,网织红细胞计数30%(20,400/微升),白细胞计数20,300/微升,血小板计数16.9×10⁴/微升,间接胆红素9.4毫克/分升。直接和间接抗人球蛋白试验均呈阳性,并鉴定出IgG自身抗体。骨髓穿刺显示细胞增多,巨核细胞增多,红系增生。患者被诊断为特发性温抗体型AIHA,入院第一天即开始使用泼尼松龙80毫克/天进行治疗,但溶血伴网织红细胞减少进展迅速,患者处于急性生命危险状态,入院第三天出现多器官功能衰竭(急性肾衰竭、成人呼吸窘迫综合征、充血性心力衰竭、肝功能障碍、横纹肌溶解)。开始进行血浆置换治疗和血液透析,并在快速输注足量血液后不久给予大剂量甲泼尼龙。开始这些治疗后,溶血情况显著改善,多器官功能衰竭得到控制,但40天后他可能因败血症导致多器官功能衰竭加重而死亡。