Background: Autoimmune hemolytic anemia (AIHA) is an uncommon disease in children. We aimed to determine the clinical characteristics and treatment outcomes in pediatric patients with AIHA.
Materials and Methods: This was a retrospective study. Pediatric patients, aged 1 month to 15 years, diagnosed with AIHA between January 2006 and December 2017 were enrolled. Demographic and laboratory data were reviewed and analyzed. Treatment response was defined as increasing > 2 g/dL of hemoglobin (Hb) and Hb value ≥ 8 g/dL.
Results: Thirty-seven patients were enrolled. The median age was 9 (3.5-13) years and 26 patients (70.3%) were female. The frequent presentations were pallor (94.6%), fever (37.8%) and jaundice (27.0%). Twenty patients (54.1%) were classified as primary AIHA. In secondary AIHA, 16 in 17 patients (94.1%) were diagnosed with systemic lupus erythematosus (SLE). All of the patients received corticosteroids (CS), the median duration were 14 (6.5-55.5) months and 29 patients (78.3%) achieved treatment response in 30 days. Three patients (8.1%) received intravenous immunoglobulin, 17 patients (45.9%) required red cell transfusions and 2 patients (5.4%) required second-line treatment. One patient in non-SLE group had relapse after treatment cessation for 15 months. At the follow-up time, 30.8% and 48.1% of the patients developed SLE at 3 and 5 years, respectively. The significant factor associated with SLE was older age at diagnosis [adjusted Odds ratio 1.22 (1.02-1.48), p-value 0.034].
Conclusion: AIHA is an uncommon cause of hemolytic anemia in children. Pediatric patients with AIHA had well response to CS and approximately half of the patients required transfusions. Older age at diagnosis was the significant risk factor to develop SLE.
Keywords: autoimmune hemolytic anemia, pediatric patients, clinical characteristics, treatment outcome
