Spectrum and outcome of autoimmune hemolytic anemia in children: single-center experience in 10 years

Abstract

Background Autoimmune hemolytic anemia (AIHA) is a rare disease in children. Little is known about its spectrum and outcome. The objective of this study was to analyze the clinical and hematological presentation, treatment response, and disease outcome in a cohort of children and adolescents whose initial presentation was acute AIHA. Patients and methods This was a retrospective analysis of children who presented with acute AIHA over 10 years. These cases presented to the Hematology Department, Children Hospital, Ain Shams University, Cairo, Egypt, from 2002 to 2012. Thirty-two children and adolescents were assessed for initial presentation, treatment received, and its response and disease outcome. Results In all, 78% of the patients were females. All presented with pallor, 81% with jaundice, and 34% with concomitant acute nonspecific febrile illness. Positive serology against the Epstein-Barr virus, cytomegalovirus, or mycoplasma was shown by 37.5% of the patients. Hepatosplenomegaly on initial presentation or evolving during the first year occurred in 53% of the patients. The direct antiglobulin test was negative in 6% and reticulocytopenia occurred in 12% of the patients. Ninety-four percent of patients received corticosteroids as initial therapy and 28% needed additional treatment subsequently. Complete remission (CR) at 1 month was achieved in 46.9% of patients and was highly correlated with disease outcome at 3, 12 months, and at last follow-up. Evans' syndrome was diagnosed in 50% of cases and was significantly associated with a lower subsequent CR rate. Conclusion CR of AIHA at 1 month was predictive of subsequent CR. Concordance with Evans' syndrome occurred in half of the cases with less favorable outcome.