Survival in autoimmune hemolytic anemia remains poor, results from a nationwide cohort with 37 years of follow-up

Introduction: Autoimmune hemolytic anemia (AIHA) is considered a chronic disease, with an overall good prognosis. However, recent reports indicate pre-mature mortality. Causes of death have not been evaluated previously. Methods: In a nationwide setting, we identified all patients with warm type AIHA or cold agglutinin disease (CAD), and age–sex-matched comparators from Denmark, 1980–2016. We estimated overall survival and cause-specific mortality from anemia, infection, cardiovascular causes, hematological or solid cancer, bleeding, or other causes, using cumulative incidence proportions. Results: We identified 1460 patients with primary AIHA, 1078 with secondary AIHA, 112 with CAD, and 130 801 comparators. One-year survival and median survival were, 82.7% and 9.8 years for primary AIHA, 69.1% and 3.3 years for secondary AIHA, and 85.5% and 8.8 years for CAD. Prognosis was comparable to the general population only in patients with primary AIHA below 30 years. In all other age and subgroups, the difference was considerable. Cumulated cause-specific mortality at 1 year was increased among patients versus comparators. Discussion: All groups of autoimmune hemolytic anemia are associated with increased overall and cause-specific mortality compared to the general population. This probably reflects unmet needs in both treatment and follow-up programs.