A new Danish study reports increased overall and cause-specific mortality among patients with warm-type autoimmune hemolytic anemia (AIHA) or cold agglutinin disease (CAD) after a 37-year nationwide cohort follow-up. The study, published in the European Journal of Haematology, represents the longest cohort observation time of patients with AIHA and the largest cohort assessed in terms of causes of death for these patients.

“We identified 1460 patients with primary AIHA, 1078 with secondary AIHA, 112 with CAD, and 130,801 comparators,” the authors wrote. “Patients with all types of immune hemolysis have a higher overall mortality and cause-specific mortality than age-sex matched general population comparators.”

The cohort of 2650 Danish patients was followed from 1980 to 2016, and the results showed that patients with primary and secondary AIHA and CAD had reduced survival compared to the general population. However, those who were diagnosed with primary AIHA before 30 years of age had a survival comparable with the comparators.

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Of particular concern is the finding that the prognosis only slightly improved over the 37-year study period. The median survival of patients with CAD was 8.8 years, and the primary causes of death in the first year for these patients included cardiovascular disease, hematological cancer, and infection. Cardiovascular causes of death were the most common at 5 and 10 years after diagnosis.

The authors concluded that mortality in these diseases is significantly higher than that of the general population, in particular during the first year postdiagnosis, and it does not improve for most patients. The results suggest significant unmet needs in terms of patient management in AIHA and CAD.


Hansen DL, Möller S, Frederiksen H. Survival in autoimmune hemolytic anemia remains poor, results from a nationwide cohort with 37 years of follow-up. Eur J Haematol. Published online March 11, 2022. doi:10.1111/ejh.13764