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

Br J Haematol. 2022 Jan;196(1):45-62. doi: 10.1111/bjh.17648. Epub 2021 Jun 30.

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.

Keywords: autoimmune haemolytic anaemia; autoimmunity; haematopoietic stem cell transplantation; primary immunodeficiency; rituximab.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Anemia, Hemolytic, Autoimmune / diagnosis
  • Anemia, Hemolytic, Autoimmune / epidemiology
  • Anemia, Hemolytic, Autoimmune / etiology*
  • Anemia, Hemolytic, Autoimmune / therapy*
  • Clinical Decision-Making
  • Combined Modality Therapy
  • Disease Management
  • Disease Susceptibility
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Incidence
  • Postoperative Care
  • Prognosis
  • Retreatment
  • Risk Assessment
  • Risk Factors
  • Transplantation, Homologous
  • Treatment Outcome