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

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.
Competing Interests: Declaration of Competing Interest WB: Consultancy for Alexion, Bioverativ, True North Therapeutics and Apellis, travel support and lecture honoraria from Bioverativ, Alexion and Novartis. SB: Research support from Mundipharma, travel support from Alexion and Apellis, lecture honoraria from Bioverativ and Janssen-Cilag, consultancy for Apellis, Bioverativ, Momenta Pharmaceuticals and True North Therapeutics. CB: Research support from Alexion and Bioverativ, honoraria from Alexion, Apellis and Bioverativ. MG: Personal fees from Ionis/Akcea, Alnylam, Prothena, Celgene, Janssen, Annexon, Appellis, Amgen, Medscape, Physicians Education Resource, Research to Practice, personal fees for Data Safety Monitoring board from Abbvie, grants and personal fees from Spectrum, speaker fees from Teva, Johnson and Johnson, Medscape, DAVA oncology; Advisory Board for Pharmacyclics and for Proclara outside the submitted work; Royalties from Springer Publishing; Grant Funding Amyloidosis Foundation; International Waldenstrom Foundation. NCI SPORE MM SPORE 5P50 CA186781-04. AH: Honoraria: Alexion, Apellis, Bioverativ, Novartis, Ra Pharma, Regeneron, Samsung. QAH: Research support from Alexion, honoraria for lecturing or advisory work from Alexion, Bioverativ, Grifols, Novartis and Shire. UJ: Research support from Bioverativ, Roche; honoraria for lecturing or advisory boards from Abbvie, Bioverativ, Gilead, Janssen, Mundipharma, Novartis, Roche, Sandoz. BJ: Reimbursement related to scientific presentations and scientific advice from TrueNorth Therapeutics and Bioverativ. DK: Research: Actelion (Syntimmune), Agios, Alnylam, Amgen, Argenx, Bristol Myers Squibb (BMS), Kezar, Principia, Protalex, Rigel, Takeda (Bioverativ). Consulting: Actelion (Syntimmune), Agios, Alnylam, Amgen, Argenx, Bristol Myers Squibb (BMS), Caremark, Daiichi Sankyo, Dova, Kyowa-Kirin, Merck Sharp Dohme, Momenta, Novartis, Pfizer, Platelet Disorder Support Association, Principia, Protalex, Protalix, Rigel, Sanofi, Genzyme, Shionogi, Shire, Takeda (Bioverativ), UCB, Up-To-Date, Zafgen. MMi: Research support from Roche, consultancy (advisory boards and symposia) for Alexion, consultancy (advisory board) for Bioverativ. MMo: Consultancy for Abbvie, Roche, Janssen, Gilead, travel support and honoraria from Abbvie, Roche, Janssen, Gilead. AR: Research support from Alexion and Roche, travel support from Alexion and AbbVie, lecture honoraria from Alexion, Roche and Novartis, consultancy for Alexion, Bioverativ, Novartis, and True North Therapeutics. SZ: Unrestricted grant from Shire, consultancy for Alexion, Bioverative, Shire.
(Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.)