Online Articles: Original ArticlesAutoimmune Hemolytic Anemia in Children Mayo Clinic ExperienceSankaran, Janani MBBS; Rodriguez, Vilmarie MD; Jacob, Eapen K. MD; Kreuter, Justin D. MD; Go, Ronald S. MDAuthor Information Divisions of *Hematology †Pediatric Hematology-Oncology ‡Transfusion Medicine, Mayo Clinic, Rochester, MN The authors declare no conflict of interest. Reprints: Ronald S. Go, MD, Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (e-mail: [email protected]). Received September 29, 2015 Accepted January 23, 2016 Journal of Pediatric Hematology/Oncology: April 2016 - Volume 38 - Issue 3 - p e120-e124 doi: 10.1097/MPH.0000000000000542 Buy Metrics Abstract We studied 35 pediatric patients with autoimmune hemolytic anemia seen at Mayo Clinic from 1994 to 2014. The median age was 10.0 years and 65.7% were males. Most had warm antibodies (80.0%) and some secondary to viral (14.3%) or autoimmune disorders (31.4%). Seven (20.0%) patients presented with Evans syndrome, 3 of whom also had common variable immunodeficiency. The median hemoglobin at diagnosis was 6.1 g/dL and 62.8% patients required red cell transfusions. The severity of anemia was worse among children below 10 years (median 5.5 vs. 7.0 g/dL, P=0.01). Steroid was the initial treatment for 88.5% patients, with overall response rate of 82.7% (68.5% complete, 14.2% partial) and median response duration of 10.7 months (range, 0.2 to 129.7+ mo). After median follow-up of 26.6 months, 8 (22.8%) patients relapsed. Salvage treatments included splenectomy, intravenous immunoglobulin, rituximab, and mycophenolate mofetil. Infectious complications occurred in 9 (25.7%) patients and 1 patient died of cytomegalovirus infection. Four patients had cold agglutinin disease and 3 (75.0%) responded to steroids. Autoimmune hemolytic anemia is a rare disorder in pediatric population and most respond well to steroids regardless of the type of antibody. Infectious complications are common and screening for immunodeficiency is recommended among those with Evans syndrome. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.