Pathogenesis, risk factors and therapeutic options for autoimmune haemolytic anaemia in the post-transplant setting.

Abstract

Autoimmune haemolytic anaemia (AIHA) is a rare complication of allogeneic haematopoietic stem cell transplantation (HSCT), observed with an incidence of 1-5%. Paediatric age, diagnosis of non-malignant disease, lympho-depleting agents in the conditioning regimen, use of unrelated donor, graft versus host disease and infections have been associated with a higher risk of AIHA post HSCT. Post-HSCT AIHA is associated with high mortality and morbidity, and it is often very difficult to treat. Steroids and rituximab are used with a response rate around 30-50%. These and other therapeutic strategies are mainly derived from data on primary AIHA, although response rates in post-HSCT AIHA have been generally lower. Here we review the currently available data on risk factors and therapeutic options. There is a need for prospective studies in post-HSCT AIHA to guide clinicians in managing these complex patients.