Efficacy and safety of second-line treatment in Thai patients with primary warm-type autoimmune hemolytic anemia

Objectives:: To investigate the efficacy and safety of second-line treatment in Thai patients with primary warm-type autoimmune hemolytic anemia (AIHA) that failed corticosteroid treatment. Methods:: This descriptive retrospective study included patients aged >14 years who were diagnosed with and treated for primary warm-type AIHA at the Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand, during January 2007 to December 2016. All 54 included patients failed first-line corticosteroid treatment after which second-line treatment was prescribed. Baseline clinical characteristics, laboratory results at diagnosis and at start of second-line treatment, type of second-line treatment, treatment outcome, and complications of treatment including death were collected. Results:: Included patients had a mean age at onset of 55.8 years (14.5-87.4) and 83.3% of patients were female. Most patients (63%) were refractory to steroids, and the rest of them relapsed while on steroids. The second-line medications were azathioprine (61.1%), cyclophosphamide (31.5%), chlorambucil (1.9%), danazol (3.7%), and rituximab (1.9%), with respective response rates of 78.8%, 58.8%, 1/1 patient, 2/2 patients, and 0/1 patient. Strong positive direct Coombs' test (3+-4+) was the only predictive factor of treatment response (p = 0.008). Males had better relapse-free survival than females (not reached vs. 20.6 months) (p = 0.023). Approximately 40% of the patients who responded to second-line treatment relapsed at a median of 7.4 months. Conclusion:: Immunosuppressive drugs are the most common second-line treatment for primary warm-type AIHA in Thailand; however, relapse was common. Additional therapies are needed to reduce the relapse rate and prolong remission.