Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea
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 t...
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The Korean Pediatric Society
2025-07-01
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| Series: | Clinical and Experimental Pediatrics |
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| Online Access: | http://e-cep.org/upload/pdf/cep-2024-02026.pdf |
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| author | Young Dai Kwon Eun Sun Jung Yeon Jung Lim |
| author_facet | Young Dai Kwon Eun Sun Jung Yeon Jung Lim |
| author_sort | Young Dai Kwon |
| collection | DOAJ |
| description | 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 6 with neonatal alloimmune hemolytic anemia, 2 who were treated at other centers, and 2 with transient bone marrow suppression due to a viral infection. Thus, this study retrospectively analyzed the treatment responses of 8 patients with AIHA. Results The median age at diagnosis was 5.2 years (range, 2.3–11.8 years); 62.5% (5 of 8) were male. Median hemoglobin (Hb) at diagnosis was 6.3 g/dL (range, 3.4–9.5 g/dL), median reticulocyte index was 6.53% (range, 1.64%–22.07%), median total bilirubin was 2.75 mg/dL (range, 0.98–7.23 mg/dL), and median lactate dehydrogenase was 1,662 U/L (range, 790–2,921 U/L). All haptoglobin levels were <10 mg/dL. Treatments included steroids (8 of 8), red blood cell transfusions (5 of 8), and intravenous immunoglobulins (2 of 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 days (range, 9.0–22.0 days). Steroids were administered for a median 37.5 days (range, 14.0–119.0 days). Excluding one patient later diagnosed with systemic lupus erythematosus, no relapses occurred during the 3- to 19-month follow-up period. Conclusion Patients with pediatric AIHA showed relapsefree 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. |
| format | Article |
| id | doaj-art-e9c0f2d629f842c99dbe8c5848eb3eed |
| institution | Kabale University |
| issn | 2713-4148 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | The Korean Pediatric Society |
| record_format | Article |
| series | Clinical and Experimental Pediatrics |
| spelling | doaj-art-e9c0f2d629f842c99dbe8c5848eb3eed2025-08-20T03:28:47ZengThe Korean Pediatric SocietyClinical and Experimental Pediatrics2713-41482025-07-0168752252910.3345/cep.2024.0202620125555813Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from KoreaYoung Dai Kwon0Eun Sun Jung1Yeon Jung Lim2 Department of Pediatrics, Chungnam National University Hospital, Daejeon, Korea Department of Pediatrics, Chungnam National University Hospital, Daejeon, Korea Department of Pediatrics, Chungnam National University Hospital, Daejeon, KoreaBackground 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 6 with neonatal alloimmune hemolytic anemia, 2 who were treated at other centers, and 2 with transient bone marrow suppression due to a viral infection. Thus, this study retrospectively analyzed the treatment responses of 8 patients with AIHA. Results The median age at diagnosis was 5.2 years (range, 2.3–11.8 years); 62.5% (5 of 8) were male. Median hemoglobin (Hb) at diagnosis was 6.3 g/dL (range, 3.4–9.5 g/dL), median reticulocyte index was 6.53% (range, 1.64%–22.07%), median total bilirubin was 2.75 mg/dL (range, 0.98–7.23 mg/dL), and median lactate dehydrogenase was 1,662 U/L (range, 790–2,921 U/L). All haptoglobin levels were <10 mg/dL. Treatments included steroids (8 of 8), red blood cell transfusions (5 of 8), and intravenous immunoglobulins (2 of 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 days (range, 9.0–22.0 days). Steroids were administered for a median 37.5 days (range, 14.0–119.0 days). Excluding one patient later diagnosed with systemic lupus erythematosus, no relapses occurred during the 3- to 19-month follow-up period. Conclusion Patients with pediatric AIHA showed relapsefree 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.http://e-cep.org/upload/pdf/cep-2024-02026.pdfanemiaautoimmune hemolytic anemiapediatricssteroids |
| spellingShingle | Young Dai Kwon Eun Sun Jung Yeon Jung Lim Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea Clinical and Experimental Pediatrics anemia autoimmune hemolytic anemia pediatrics steroids |
| title | Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea |
| title_full | Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea |
| title_fullStr | Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea |
| title_full_unstemmed | Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea |
| title_short | Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea |
| title_sort | treatment and clinical outcomes of pediatric autoimmune hemolytic anemia real world single center data from korea |
| topic | anemia autoimmune hemolytic anemia pediatrics steroids |
| url | http://e-cep.org/upload/pdf/cep-2024-02026.pdf |
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