Quarterly Medical ReviewWarm autoimmune hemolytic anemia: Advances in pathophysiology and treatment
Section snippets
Pathophysiology
The pathogenesis of wAIHA is a complex multistep process involving not only the auto-antibodies directed towards RBC's membrane antigens but also various effectors of the immune system including the complement system, macrophages as well as B and T-cells [7]. Whereas the mechanisms leading to hemolysis are partially elucidated (antibody-dependent cell-mediated cytotoxicity and complement dependent cytotoxicity are primarily involved), the mechanisms leading to the breakdown of self-tolerance
Treatment of wAIHA
The management of wAIHA has long been and still is mainly empirical or based on retrospective uncontrolled studies [32], [33]. It is only recently that the first 2 prospective studies including one randomized-controlled study focused on adult's wAIHA have been reported.
Systemic lupus erythematosus-associated wAIHA
Autoimmune hemolytic anemia occurs in approximately 5 to 10% of patients with SLE and is more frequently observed in patient of African ancestry [58]. wAIHA may be the sole presenting sign of the disease and may precede the appearance of other disease manifestations by several years. wAIHA in the setting of SLE is often associated with the presence of antiphospholipid antibodies with or without a definite antiphospholipid syndrome and has been associated with a higher risk of thrombosis [58].
Conclusion
wAIHA has long been viewed as a rare autoimmune disease that could be treated empirically mainly by corticosteroids on a long-term. Thanks to some informative observational studies and efforts made in the classification of wAIHA (primary versus secondary) the natural history and prognosis of the disease is henceforth better known. Based on retrospective and more recently on prospective trials, rituximab has emerged in the last decade as the preferred second-line option prior to splenectomy. As
Disclosure of interest
the author has received research support from Roche for a clinical trial in wAIHA.
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