Pediatric autoimmune hemolytic anemia: A single-center retrospective study

Background

Autoimmune hemolytic anemia (AIHA) is a rare, life-threatening disease in pediatrics. This article describes the clinical features, diagnostic workup, treatment and outcome in patients with AIHA.

Method

Medical charts of under 18-year-old patients with AIHA treated at a tertiary Brazilian institution from 2006 to 2021 were retrospectively reviewed. Data analysis was primarily descriptive, using medians, interquartile ranges, and categorical variables presented as absolute frequencies.

Main results

Twenty-four patients (14 female, 10 male) were evaluated in this study. The median age at diagnosis was 5.99 years (range: 0.25–17.1 years) and the median hemoglobin level was 4.85 g/dL (range: 4.17–5.57 g/dL). Most had warm antibodies (83.3 %). Twelve patients (50 %) had known underlining diseases, four (16.6 %) presented with AIHA concomitant with acute infectious diseases and three (12.5 %) had an undetermined post-vaccine association. Steroids and intravenous immunoglobulin were first-line therapy in 23 cases. Seven patients (29.1 %) required second and third-line treatments (rituximab, cyclophosphamide and splenectomy). The median follow-up period was 4.4 years (range: 1.0–6.7 years). Thirteen patients (54.1 %) were discharged, five cases (20.8 %) were lost to follow-up and no patient died. The median age for the six remaining patients was 11.53 years (8.5–14.7) with all of them having complete responses with no further therapies.

Conclusion

Most cases of AIHA are secondary to an underlying systemic disease or have a possible correlation with infections/vaccines and respond to steroids. The second and third-line therapies for refractory and relapse cases remain a dilemma. A prospective, multicenter study is essential to address the best therapeutic combinations.