Autoimmune Hemolytic Anemia in the Pediatric Setting.
Autoimmune Hemolytic Anemia in the Pediatric Setting.
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DOI：
10.3390/jcm10020216
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发表时间：
2021-01-09
影响因子：
3.9
通讯作者：
Kalfa TA
中科院分区：
文献类型：
--
作者：
Voulgaridou A;Kalfa TA
自身免疫性溶血性贫血（AIHA）是一种罕见的儿童疾病，表现出不同的严重程度。最常见的是，热反应性IgG抗体在37℃时结合红细胞，主要由脾巨噬细胞诱导调理和吞噬，引起温性AIHA （w-AIHA）。感染后冷反应抗体也可导致患者暴露于低温后溶血，由于IgM自身抗体引起冷凝集素综合征（CAS），或由于非典型IgG自身抗体结合靶红细胞抗原并在4°C下固定补体而引起阵发性冷血红蛋白尿（PCH）。冷反应抗体主要在补体激活后诱导血管内溶血。直接抗球蛋白试验（DAT）是AIHA诊断的金标准；然而，高达11%的温暖型AIHA患者出现了DAT阴性结果，这强调了对表型与免疫介导溶血性贫血相容的病例进行进一步评估的必要性，尽管DAT阴性。及时的支持性护理，对w-AIHA进行类固醇治疗，并在必要时对有症状或快速发展的贫血进行输血，对于取得积极结果至关重要。儿童w-AIHA通常继发于潜在的免疫失调综合征，因此，在开始使用免疫抑制剂治疗之前，建议在就诊时对此类疾病进行筛查，以确定预后并优化可能使用新型靶向药物的长期管理。
Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient’s exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Cold-reactive antibodies mainly induce intravascular hemolysis after complement activation. Direct antiglobulin test (DAT) is the gold standard for AIHA diagnosis; however, DAT negative results are seen in up to 11% of warm AIHA, highlighting the need to pursue further evaluation in cases with a phenotype compatible with immune-mediated hemolytic anemia despite negative DAT. Prompt supportive care, initiation of treatment with steroids for w-AIHA, and transfusion if necessary for symptomatic or fast-evolving anemia is crucial for a positive outcome. w-AIHA in children is often secondary to underlying immune dysregulation syndromes and thus, screening for such disorders is recommended at presentation, before initiating treatment with immunosuppressants, to determine prognosis and optimize long-term management potentially with novel targeted medications.
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