Department of Pediatrics, Chungbuk National University Hospital, Cheongju-si, Chungcheongbuk-do, Republic of Korea.
Department of Pediatrics, Konyang University Hospital, Daejeon, Korea, Republic of Korea.
BMC Pediatr. 2024 Jan 20;24(1):69. doi: 10.1186/s12887-024-04546-z.
The administration of high-dose intravenous immunoglobulin (IVIG) is a standard treatment for the management of Kawasaki disease (KD). IVIG is known to be a highly effective and safe treatment.
We report the development of hemolytic anemia in seven children receiving repeated doses of IVIG. The children were aged 3-44 months and included 4 girls and 3 boys. All children received 10% IVIG and a second course of immunoglobulin because they did not respond to the first course of immunoglobulin. Two received high-dose aspirin (50 mg/kg), and five received low-dose aspirin (5 mg/kg). Two patients required additional methylprednisolone pulse therapy (30 mg/kg) after the second dose of immunoglobulin, and three patients received oral prednisolone therapy for defervescence. Three patients showed coronary artery dilation during hospitalization and normalized within two months. Pretreatment hemoglobin averaged 11.3-14.2 g/dL, and post-hemolytic anemia hemoglobin ranged from 7.4 to 9.6 g/dL, with a difference of 1.7-6.8 g/dL. Reticulocytes were increased to 3.3-13.2%. Peripheral blood smears showed normochromic normocytic anemia, and anisopoikilocytosis. All children were positive for warm-type antibodies with IgG+, C3d- in direct antiglobulin test, and the blood group was A + in five and B + in two. None of the patients received immunomodulatory therapy or red blood cell transfusions. They were followed for a year and all recovered.
Especially, in non-O blood group KD patients who are refractory to initial IVIG and require a second dose of IVIG or 10% formulation the possibility of immune hemolytic anemia should be carefully considered, and close follow-up should be maintained after therapy.
大剂量静脉注射免疫球蛋白（IVIG）的给药是川崎病（KD）治疗的标准方法。IVIG 已被证明是一种高度有效和安全的治疗方法。
我们报告了七例接受重复 IVIG 剂量的儿童发生溶血性贫血的情况。这些儿童的年龄为 3-44 个月，包括 4 名女孩和 3 名男孩。所有儿童均接受 10% IVIG 和第二疗程的免疫球蛋白治疗，因为他们对第一疗程的免疫球蛋白没有反应。其中 2 名儿童接受高剂量阿司匹林（50mg/kg）治疗，5 名儿童接受低剂量阿司匹林（5mg/kg）治疗。2 名患者在第二剂免疫球蛋白后需要额外的甲基强的松龙脉冲治疗（30mg/kg），3 名患者因退热而接受口服泼尼松龙治疗。3 名患者在住院期间出现冠状动脉扩张，两个月内恢复正常。预处理血红蛋白平均为 11.3-14.2g/dL，溶血性贫血后血红蛋白范围为 7.4-9.6g/dL，相差 1.7-6.8g/dL。网织红细胞增加至 3.3-13.2%。外周血涂片显示正细胞正色素性贫血和异形红细胞增多症。所有儿童的直接抗球蛋白试验均为温型抗体阳性，IgG+C3d-，血型在 5 例中为 A+，在 2 例中为 B+。所有患者均未接受免疫调节治疗或红细胞输注。他们随访了一年，均已康复。
特别是在初始 IVIG 无效且需要第二剂 IVIG 或 10%制剂的非 O 血型 KD 患者中，应仔细考虑免疫性溶血性贫血的可能性，并在治疗后密切随访。
