Clinical Analysis Of Childhood Autoimmune Hemolytic Anemia In 68 Cases

Objective: To study the clinical features and treatment outcomes of childhood autoimmune hemolytic anemia(AIHA).Methods: Totally 68 cases of childhood AIHA were enrolled. Among them, Evans syndrome(ES) accounted for 20 cases(29.4%). They were all treated by glucocorticoid, some of them also received other therapies such as intravenous immunoglobulin(IVIG) or immunosuppressor. We recorded the clinical presentations, laboratory findings, and treatment outcomes. Meanwhile, the degrees of anemia, curative effects were compared between different groups.Results:(1)Data show that Primary AIHA accounted for 39.7% of all patients, while secondary AIHA accounted for 60.3%. Among them, ES accounted for 20 cases(29.4%). 63.2% cases were 3 years of age or younger.(2)Average hemoglobin(Hb) was lower in the 1-year or below age group than in the above 1-year age group, combined-antibody group than single-antibody group, and IgM-contained group than non–IgM-contained group(P<0.05 for all).(3)For free plasma Hb, there is no statistical difference between the 1-year or below age group and the above 1-year age group, combined-antibody group and single-antibody group, IgM-contained group and non–IgM-contained group, AIHA-only group and ES group(P>0.05 for all).(4)In AIHA-only patients, the median indirect bilirubin was higher than that in ES patients(P<0.05).(5)The duration of therapy in the ES group was longer than that in the AIHA-only group(P<0.05), while the duration of therapy was not significantly impacted by age, IVIG, or types of serologic antibody groups.(6)With glucocorciod therapy, all of the 45 patients with complete follow-up data achieved complete remission(CR). During the follow-up period, 29 cases(29/45, 64.4%) remained in continuous CR. In total, 35.6% of patients relapsed after first CR and 56.3% of them still showed good response to glucocorticoid after relapse. There was no difference in the relapse rate between the AIHA-only group and ES group, 1-year or below age group and the above 1-year age group, combined-antibody group and single-antibody group, IgM-contained group and non–IgM-contained group, the IVIG-treatment group and the non-IVIG-treatment group(P>0.05).Conclusions: The severity of anemia correlates with age and serologic types of direct antiglobulin test. Glucocorticoid is efficacious for AIHA regardless of whether it is a first attack or relapse in this cohort of young patients. ES needs longer treatment duration. IVIG does not improve the outcome of AIHA.