Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting

Ulrich Jäger, Wilma Barcellini, Catherine M. Broome, Morie A. Gertz, Anita Hill, Quentin A. Hill, Bernd Jilma, David J. Kuter, Marc Michel, Marco Montillo, Alexander Röth, Sacha S. Zeerleder, Sigbjørn Berentsen

Research output: Contribution to journalReview article

15 Scopus citations

Abstract

Autoimmune hemolytic anemias (AIHAs) are rare and heterogeneous disorders characterized by the destruction of red blood cells through warm or cold antibodies. There is currently no licensed treatment for AIHA. Due to the paucity of clinical trials, recommendations on diagnosis and therapy have often been based on expert opinions and some national guidelines. Here we report the recommendations of the First International Consensus Group, who met with the aim to review currently available data and to provide standardized diagnostic criteria and therapeutic approaches as well as an overview of novel therapies. Exact diagnostic workup is important because symptoms, course of disease, and therapeutic management relate to the type of antibody involved. Monospecific direct antiglobulin test is considered mandatory in the diagnostic workup, and any causes of secondary AIHA have to be diagnosed. Corticosteroids remain first-line therapy for warm-AIHA, while the addition of rituximab should be considered early in severe cases and if no prompt response to steroids is achieved. Rituximab with or without bendamustine should be used in the first line for patients with cold agglutinin disease requiring therapy. We identified a need to establish an international AIHA network. Future recommendations should be based on prospective clinical trials whenever possible.

Original languageEnglish (US)
Article number100648
JournalBlood Reviews
Volume41
DOIs
StatePublished - May 2020

Keywords

  • Autoimmune hemolytic anemia
  • Cold agglutinins
  • Diagnosis–Treatment
  • Warm agglutinins

ASJC Scopus subject areas

  • Hematology
  • Oncology

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    Jäger, U., Barcellini, W., Broome, C. M., Gertz, M. A., Hill, A., Hill, Q. A., Jilma, B., Kuter, D. J., Michel, M., Montillo, M., Röth, A., Zeerleder, S. S., & Berentsen, S. (2020). Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood Reviews, 41, [100648]. https://doi.org/10.1016/j.blre.2019.100648