Clinical Profile and Outcome of Childhood Autoimmune Hemolytic Anemia: A Single Center Study

摘要

Objective To analyze clinical and laboratory parameters, and treatment outcomes of children with autoimmune hemolytic anemia (AIHA). Methods Retrospective analysis of 50 children aged 0–18 years. Monospecific direct antiglobulin test (DAT) and investigations for secondary causes were performed. Disease status was categorized based on Cerevance criteria. Results Median (range) age at diagnosis was 36 (1.5–204) months. AIHA was categorized as cold (IgM+,C3+/cold agglutinin+) (35%), warm (IgG+ with/without C3+) (28%), mixed (IgG+, IgM+, C3+) (15%) and paroxysmal cold hemoglobinuria (4%). Primary AIHA accounted for 64% cases. Treatment modalities included steroid (66%), intravenous immunoglobulin (IVIg) (4%), steroid+IVIg (4%), and steroid+rituximab (4%). Treatment duration was longer for secondary AIHA than primary (11 vs 6.6 months, P <0.02) and in patients needing polytherapy than steroids only (13.3 vs 7.5 months, P <0.006). During median (range) follow-up period of 73 (1–150) months, 29 (58%) remained in continuous complete remission, 16 (32%) remained in complete remission. Conclusion Infants with AIHA have a more severe presentation. Monospecific DAT and a thorough search for an underlying cause help optimize therapy in most patients of AIHA.