Clin Exp Pediatr. 2025 Apr 16. doi: 10.3345/cep.2024.02026. Online ahead of print.
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.
PMID:40241594 | DOI:10.3345/cep.2024.02026
