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

Jäger, Ulrich; Barcellini, Wilma; Broome, Catherine M; Gertz, Morie A; Hill, Anita; Hill, Quentin A; Jilma, Bernd; Kuter, David J; Michel, Marc; Montillo, Marco; Röth, Alexander; Zeerleder, Sacha Sergio; Berentsen, Sigbjørn (2020). Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood reviews, 41, p. 100648. Elsevier 10.1016/j.blre.2019.100648

[img] Text
Diagnosis and treatment.pdf - Published Version
Restricted to registered users only
Available under License Publisher holds Copyright.

Download (762kB) | Request a copy

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.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Unit Childrens Hospital > Forschungsgruppe Hämatologie (Erwachsene)

UniBE Contributor:

Zeerleder, Sacha Sergio


600 Technology > 610 Medicine & health








Pierrette Durand Lüthi

Date Deposited:

08 Jan 2020 09:15

Last Modified:

20 May 2020 01:31

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Autoimmune hemolytic anemia Cold agglutinins Diagnosis–Treatment Warm agglutinins





Actions (login required)

Edit item Edit item
Provide Feedback