Autoimmune hemolytic anemia is an autoimmune disorder resulting in the production of antibodies directed against red blood cell antigens, causing shortened erythrocyte survival. The disorder is an acquired hemolysis, and can be idiopathic or secondary to other autoimmune disorders, malignancies, or infections. Building on the recommendations that emerged from the First International Consensus Meeting on AIHA in 2019, FWGBD will provide web-based resources that focus on optimal treatment and management recommendations for patients with AIHA; bolster knowledge of medical complications that patients with AIHA may have; and provide cross-functional provider team management of medical complications for patients with AIHA.
Ask-the-Experts is a resource for healthcare providers that enables medical professionals to submit a question to the Foundation's Board and Medical Advisory Committee members to receive their opinion on treatment and management of medical conditions. These researchers and educators at the top of their respective fields offer their perspectives on managing AIHA in complex cases.
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FWGBD has collected the best-in-class resources to guide providers in their diagnosis, treatment, and management of patients with AIHA. The resources, gathered with input from clinical experts, is intended to support providers in developing their knowledge base related to this often difficult to manage disease. Providers, especially those outside hematology, may have knowledge and practice gaps with regard to appropriate diagnosis of AIHA, given that diagnosis is based on set of highly specific biological changes and on a positive direct Coombs test. Because the course of the disorder varies across populations and may be chronic or idiopathic, the treatment protocols may also vary, but primarily involve immune modulation with corticosteroids and other agents, including a growing focus on complement inhibitors. Realizing that AIHA can carry a significant patient burden, the resources were collected with a patient-focused lens to help evaluate the impact of various disease management options on quality of life.
- International Consensus Report on AIHA Provider-driven medical summary on the treatment and management of AIHA, drawn from physicians worldwide
- New England Journal of Medicine Treatment Guide on AIHA The NEJM has curated a set of resources for providers
- Autoimmune Hemolytic Anemia Resource Center The CDC’s NHLBI collected resources of evergreen information for a good primer for providers
- Autoimmune Hemolytic Anemia in Emergency Medicine Questions & Answers Medscape features a short summary of ITP from diagnosis to treatment to ongoing management
For most patients, the first-line treatment of warm autoimmune hemolytic anemia is corticosteroids, which can be associated with side effects that may lead to patients stopping or reducing therapy, despite the overall benefits. In addition to managing medication, successful treatment of AIHA involves open communication and support between provider and patient in order to help the patients navigate the burden of managing the disease. With proper support, patients and providers may navigate the difficult journey of ongoing management and enhance the patient’s quality of life. The following articles drawn from leading academic journals offer insight from other providers’ research to help inform care of your own patients:
- Warm Autoimmune Hemolytic Anemia. Brodsky R. N Engl J Med.2019; 381:647-654, DOI: 10.1056/NEJMcp1900554
- The Changing Landscape of Autoimmune Hemolytic Anemia. Barcellini W, Fattizzo B. Front Immunol. 2020 Jun 3; 11:946. doi: 10.3389/fimmu.2020.00946. PMID: 32655543; PMCID: PMC7325906.
- Defining autoimmune hemolytic anemia: a systematic review of the terminology used for diagnosis and treatment. Hill QA, Hill A, Berentsen S. Blood Adv. 2019 Jun 25; 3(12):1897-1906. doi: 10.1182/bloodadvances.2019000036. PMID: 31235526; PMCID: PMC6595261.
- Autoimmune hemolytic anemia: current knowledge and perspectives. Michalak SS, Olewicz-Gawlik A, Rupa-Matysek J, Wolny-Rokicka E, Nowakowska E, Gil L. Immun Aging. 2020 Nov 20; 17(1):38. doi: 10.1186/s12979-020-00208-7. PMID: 33292368; PMCID: PMC7677104.
- The diagnosis and management of primary autoimmune haemolytic anaemia. Hill QA, Stamps R, Massey E, Grainger JD, Provan D, Hill A; British Society for Haematology. Br J Haematol. 2017 Feb; 176(3):395-411. doi: 10.1111/bjh.14478. Epub 2016 Dec 22. PMID: 28005293.
- “How I Treat Warm Autoimmune Hemolytic Anemia.” Drawn from ASH’s How I Treat series, this is a good summary of the key practices in treating warm autoimmune hemolytic anemia.
- How I Treat Autoimmune Hemolytic Anemia." Drawn from ASH's How I Treat series, this 2017 article provides insight into what's changed in practice, and what is still current.
- Precautions surrounding blood transfusion in autoimmune haemolytic anemias are overestimated. Yürek S, Almahallawi M, Mayer B, Pruss A, Salama A. Blood Transfus. 2015; doi: 10.2450/2015.0326-14.
- Characteristics and outcome of warm autoimmune hemolytic anemia in adults: new insights based on a single-center experience with 60 patients. Roumier M, Loustau V, Guillaud C, Languille L, Mahevas M, Khellaf M, Limal N, Noizat-Pirenne F, Godeau B, Michel M: Am J. Hematol. 2014;89:E150-155.
- Berentsen S, Sundic T. Biomed Res Int 2015:363278.
- Clinically and/or serological misleading findings surrounding immune haemolytic anaemias. Salama A. Transfus Med Hemother 2015; DOI: 10.1159/000438960.
- Immunotherapy treatments of warm autoimmune hemolytic anemia. Liu B, Gu W. Clin Dev Immunol. 2013;2013:561852. doi: 10.1155/2013/561852. Epub 2013 Sep 11. PMID: 24106518; PMCID: PMC3784078.
- Warm autoimmune hemolytic anemia: recent progress in understanding the immunobiology and the treatment. Barros MM, Blajchman MA, Bordin JO. Transfus Med Rev. 2010 Jul; 24(3):195-210. doi: 10.1016/j.tmrv.2010.03.002. PMID: 20656187.
- Clinical severity in adult warm autoimmune hemolytic anemia and its relationship to antibody specificity. Chadebech P, Loustau V, Janvier D, Languille L, Ripa J, Tamagne M, Bierling P, Djoudi R, Godeau B, Michel M, Pirenne F, Mahévas M. Haematologica. 2018 Jan; 103(1):e35-e38. doi: 10.3324/haematol.2017.175976. Epub 2017 Oct 12. PMID: 29025905; PMCID: PMC5777213.
- Evidence-based focused review of the treatment of idiopathic warm immune hemolytic anemia in adults. Crowther MA, Chan YL, Garbett IK, Lim W, Vickers MA. Blood. 2011 Oct 13; 118(15):4036-40. doi: 10.1182/blood-2011-05-347708. Epub 2011 Jul 21. PMID: 21778343.
- Treatment Options for Primary Autoimmune Hemolytic Anemia: A Short Comprehensive Review. Salama A. Transfus Med Hemother. 2015 Sep;42(5):294-301. doi: 10.1159/000438731. Epub 2015 Aug 10. PMID: 26696797; PMCID: PMC4678315.
- Treatment of autoimmune hemolytic anemias. Zanella A, Barcellini W. Haematologica. 2014;99(10):1547-1554. doi:10.3324/haematol.2014.114561
- Efficacy and safety of splenectomy in adult autoimmune hemolytic anemia. Giudice V, Rosamilio R, Ferrara I, Seneca E, Serio B, Selleri C. Open Med (Wars). 2016; 11(1):374-380. Published 2016 Nov 19. doi:10.1515/med-2016-0068.
- Steroid-Refractory Autoimmune Hemolytic Anemia with Massive Splenomegaly. Ureshino H, Miyahara M. Am J Med. 2017 Sep; 130(9):e393-e394. doi: 10.1016/j.amjmed.2017.03.017. Epub 2017 Apr 5. PMID: 28389309.
Focus on Rituximab
- Efficacy and safety of rituximab in auto-immune hemolytic anemia: a meta-analysis of 21 studies. Reynaud Q, Durieu I, Dutertre M, Ledochowski S, Durupt S, Michallet AS, Vital-Durand D, Lega JC. Autoimmun Rev 2015; 14:304-313.
- Efficacy and safety of rituximab in adults' warm antibody autoimmune haemolytic anemia: retrospective analysis of 27 cases. Bussone G, Ribeiro E, Dechartres A, Viallard JF, Bonnotte B, Fain O, Godeau B, Michel M. Am J Hematol. 2009 Mar; 84(3):153-7. doi: 10.1002/ajh.21341. PMID: 19123460.
- Rituximab therapy for autoimmune haematological diseases. Barcellini W, Zanella A. Eur J Intern Med. 2011 Jun;22(3):220-9. doi: 10.1016/j.ejim.2010.12.016. Epub 2011 Jan 31. PMID: 21570637.
- Rituximab is an effective and safe treatment of relapse in elderly patients with resistant warm AIHA. Laribi K, Bolle D, Ghnaya H, Sandu A, Besançon A, Denizon N, Truong C, Pineau-Vincent F, de Materre AB. Ann Hematol. 2016 Apr; 95(5):765-9. doi: 10.1007/s00277-016-2605-2. Epub 2016 Feb 9. PMID: 26858026.
- A multi-centre retrospective study of rituximab use in the treatment of relapsed or resistant warm autoimmune haemolytic anaemia. Maung SW, Leahy M, O'Leary HM, Khan I, Cahill MR, Gilligan O, Murphy P, McPherson S, Jackson F, Ryan M, Hennessy B, McHugh J, Goodyer M, Bacon L, O'Gorman P, Nee A, O'Dwyer M, Enright H, Saunders J, O'Keeffe D. B.r J Haematol. 2013 Oct; 163(1):118-22. doi: 10.1111/bjh.12486. Epub 2013 Aug 2. PMID: 23909468.
- A randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm auto-immune hemolytic anemia in adults (the RAIHA study). Michel M, Terriou L, Roudot-Thoraval F, Hamidou M, Ebbo M, Le Guenno G, Galicier L, Audia S, Royer B, Morin AS, Marie Michot J, Jaccard A, Frenzel L, Khellaf M, Godeau B. Am J Hematol. 2017 Jan; 92(1):23-27. doi: 10.1002/ajh.24570. Epub 2016 Nov 10. PMID: 27696475.
- Rituximab in the treatment of autoimmune haemolytic anaemia. Rodrigo C, Rajapakse S, Gooneratne L. Br J Clin Pharmacol. 2015 May;79(5):709-19. doi: 10.1111/bcp.12498. PMID: 25139610; PMCID: PMC4415708.
- Efficacy and safety of rituximab in auto-immune hemolytic anemia: A meta-analysis of 21 studies. Reynaud Q, Durieu I, Dutertre M, Ledochowski S, Durupt S, Michallet AS, Vital-Durand D, Lega JC. Autoimmun Rev. 2015 Apr; 14(4):304-13. doi: 10.1016/j.autrev.2014.11.014. Epub 2014 Dec 9. PMID: 25497766.
- Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients. Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C, Revelli N, Villa MA, Consonni D, Scaramucci L, De Fabritiis P, Tagariello G, Gaidano G, Rodeghiero F, Cortelezzi A, Zanella A. Blood. 2014; 124:2930-2936
Patients need easy-to-navigate resources to help them get started in understanding this complicated disease. These curated resources are a useful place to help your patients along their AIHA management journey.
- wAIHA Warriors wAIHA Warriors is a patient support and advocacy organization designed to share resources and create a community for patients with wAIHA and the providers who treat them.
- Resources from Platelet Disorder Support Association (PDSA) on AIHA.
- Educational Resources for Patients with AIHA St. Jude’s Research Hospital has created patient resources to help navigate ongoing treatment and management, including discussion points to ask providers.
- Resources to Help Patients Live with Autoimmune Hemolytic Anemia, from Johns Hopkins University.
- Understanding AIHA and Its Treatment, from the Indiana Hemophilia & Thrombosis Center.
- Living with Autoimmune Hemolytic Anemia Understanding AIHA, its treatment, and how to improve quality of life
These resources have been gathered in an effort to support healthcare providers, and are not intended to provide medical advice or to substitute for the judgment of healthcare professionals in the care of their patients.
We would like to thank Rigel Pharmaceuticals for their support of the development of this Resource Page, along with a few of the resources listed within.