Clinical and Laboratory Features of Autoimmune Hemolytic Anemia Associated with Immune Checkpoint Inhibitors. American journal of hematology Leaf, R. K., Ferreri, C., Rangachari, D., Mier, J., Witteles, W., Ansstas, G., Anagnostou, T., Zubiri, L., Piotrowska, Z., Oo, T. H., Iberri, D., Yarchoan, M., Salama, A., Johnson, D. B., Leavitt, A. D., Rahma, O., Reynolds, K. L., Leaf, D. E. 2019


Immune checkpoint inhibitors (ICPis) are a novel class of immunotherapeutic agents that have revolutionized the treatment of cancer; however, these drugs can also cause a unique spectrum of autoimmune toxicity. Autoimmune hemolytic anemia (AIHA) is a rare but often severe complication of ICPis. We identified 14 patients from 9 institutions across the US who developed ICPi-AIHA. The median interval from ICPi initiation to development of AIHA was 55 days (interquartile range [IQR], 22-110 days). Direct antiglobulin test (DAT) results were available for 13 of 14 patients: eight patients (62%) had a positive DAT and five (38%) had a negative DAT. The median pre-treatment and nadir hemoglobin concentrations were 11.8 g/dL (IQR, 10.2-12.9 g/dL) and 6.3 g/dL (IQR, 6.1-8.0 g/dL), respectively. Four patients (29%) had a pre-existing lymphoproliferative disorder, and two (14%) had a positive DAT prior to initiation of ICPi therapy. All patients were treated with glucocorticoids, with 3 requiring additional immunosuppressive therapy. Complete and partial recoveries of hemoglobin were achieved in 12 (86%) and 2 (14%) patients, respectively. Seven patients (50%) were re-challenged with ICPis, and one (14%) developed recurrent AIHA. Clinical and laboratory features of ICPi-AIHA were similar in DAT positive and negative patients. ICPi-AIHA shares many clinical features with primary AIHA; however, a unique aspect of ICPi-AIHA is a high incidence of DAT negativity. Glucocorticoids are an effective first-line treatment in the majority of patients with ICPi-AIHA, and most patients who are re-challenged with an ICPi do not appear to develop recurrence of AIHA. This article is protected by copyright. All rights reserved.

View details for PubMedID 30790338