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Original Article

Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea

Young Dai Kwon¹  , Eun Sun Jung¹, Yeon Jung Lim^1,2 

¹Department of Pediatrics, Chungnam National University Hospital, Daejeon, Korea
²Department of Pediatrics, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon, Korea

Correspondence Yeon Jung Lim ,Email: pedonco@cnu.ac.kr

Received: December 27, 2024; Revised: February 10, 2025   Accepted: February 24, 2025.

Abstract

Background
Autoimmune hemolytic anemia (AIHA) is rare and characterized by hemolytic anemia with a positive direct antiglobulin test result after the exclusion of other causes. While adults often relapse within 1 year of first-line steroid therapy, children generally respond well. However, current treatment approaches lack substantial evidence and are primarily expert opinion–based.
Purpose
This study aimed to contribute our single-center experience to pediatric AIHA treatment guidelines.
Methods
Between January 2012 and June 2024, 475 children were diagnosed with anemia; of them, 18 had immune hemolytic anemia, including six with neonatal alloimmune hemolytic anemia, two who were treated at other centers, and two with transient bone marrow suppression due to a viral infection. Thus, this study retrospectively analyzed the treatment responses of eight patients with AIHA.
Results
The median age at diagnosis was 5.2 (range, 2.3–11.8) years; 62.5% (5/8) were male. Median hemoglobin (Hb) at diagnosis was 6.3 (range, 3.4–9.5) g/dL, median reticulocyte index was 6.53% (range, 1.64–22.07%), median total bilirubin was 2.75 (range, 0.98–7.23) mg/dL, and median lactate dehydrogenase was 1662.0 (range, 790–2921) U/L. All haptoglobin levels were <10 mg/dL. Treatments included steroids (8/8), red blood cell transfusions (5/8), and intravenous immunoglobulins (2/8). Half of the steroid-treated patients received intravenous methylprednisolone for 1–5 days, while half received oral prednisolone (median, 1.78 [range, 0.79–3.39] mg/kg/day). The median time to age-adjusted normal Hb levels was 16.5 (range, 9–22) days. Steroids were administered for a median 37.5 (range, 14–119) days. Excluding one patient later diagnosed with systemic lupus erythematosus, no relapses occurred during the 3–19-month follow-up period.
Conclusion
Patients with pediatric AIHA showed relapse-free rapid hematological improvement and sustained steroid responses within 2 months, suggesting that systematic steroid treatment is feasible and highlighting the need for multicenter trials to establish standardized guidelines.

Keywords :Anemia, Autoimmune hemolytic anemia, Pediatrics, Steroids