Journal
BLOOD
Volume 137, Issue 10, Pages 1283-1294Publisher
AMER SOC HEMATOLOGY
DOI: 10.1182/blood.2019003808
Keywords
-
Categories
Funding
- Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (RC 2018)
Ask authors/readers for more resources
Warm autoimmune hemolytic anemia is a disease caused by increased destruction of red blood cells by IgG autoantibodies, with complex pathogenic mechanisms and unpredictable prognosis. Treatment options include steroids, splenectomy, immunosuppressants, and rituximab, but challenges exist in choosing and managing these therapies.
Warm autoimmune hemolytic anemia (wAIHA) is caused by increased erythrocyte destruction by immunoglobulin G (IgG) autoantibodies, with or without complement activation. Antibody-dependent cell-mediated cytotoxicity by macrophages/activated lymphocytes occurs in the lymphoid organs and spleen (extravascular hemolysis). The ability of the bone marrow (BM) to compensate determines clinical severity. The different pathogenic mechanisms, their complex interplay, and changes over time may explain wAIHA's great clinical heterogeneity and unpredictable course. The disease may be primary, drug induced, or associated with lymphoproliferative neoplasms, autoimmune and infectious diseases, immunodeficiencies, solid tumors, or transplants. Therapeutic interventions include steroids, splenectomy, immunosuppressants, and rituximab; the latter is increasingly used in steroid-refractory cases based on evidence from the literature and a few prospective trials. We present 5 patient case studies highlighting important issues: (1) the diagnosis and proper use of steroid therapy, (2) the concerns about the choice between rituximab and splenectomy in second-line treatment, (3) the need of periodical re-evaluation of the disease to assess the possible evolution of relapsed/refractory cases in myelodysplastic and BM failure syndromes, and (4) the difficulties in managing cases of severe/acute disease that are at high risk of relapse. Incorporating novel targeted therapies into clinical practice will be an exciting challenge in the future.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available