Takahashi Hiroyuki, Tanaka Fumiko, Sakuma Hiroyuki, Sato Mutsumi, Inaba Shoichi, Kai Sumio
Department of Pediatrics, Saiseikai Yokohama-City Southern Hospital, Yokohama, Kanagawa, Japan.
Kanagawa Red Cross Blood Center, Yokohama, Kanagawa, Japan.
Pediatr Int. 2016 Aug;58(8):744-6. doi: 10.1111/ped.12931. Epub 2016 Jun 8.
Herein is described a case of immunoglobulin M (IgM) warm autoimmune hemolytic anemia (AIHA) in a child who consequently died within 3 days of clinical onset. A previously healthy 11-year-old boy presented with fever, anemia, jaundice, and deteriorating consciousness. On direct agglutination test against group O red blood cells, agglutination was seen even at 37°C in saline, which was abolished on dithiothreitol treatment of the serum, indicating that the responsible autoantibody was IgM and had a warm-reactive capacity. A diagnosis of IgM warm AIHA was therefore made. Hemagglutination in the visceral capillaries was considered as the direct cause of organ dysfunction. The patient died due to respiratory failure. IgM warm AIHA is a very severe condition that is difficult to reverse in an advanced state. Both prompt, definite diagnosis and intervention are therefore vital to prevent severe multi-organ dysfunction in cases of IgM warm AIHA.
本文描述了一例儿童免疫球蛋白M(IgM)温抗体自身免疫性溶血性贫血(AIHA),该患儿在临床发病后3天内死亡。一名此前健康的11岁男孩出现发热、贫血、黄疸和意识恶化。在针对O型红细胞的直接凝集试验中,即使在37℃的盐水中也可见凝集现象,血清经二硫苏糖醇处理后凝集现象消失,表明致病自身抗体为IgM且具有温反应能力。因此诊断为IgM温抗体AIHA。内脏毛细血管中的血凝被认为是器官功能障碍的直接原因。患者因呼吸衰竭死亡。IgM温抗体AIHA是一种非常严重的疾病,晚期难以逆转。因此,对于IgM温抗体AIHA病例,迅速、明确的诊断和干预对于预防严重的多器官功能障碍至关重要。