Autoimmune hemolytic anemia medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2]
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Overview
Medical Therapy
The maintain of therapy for autoimmune hemolytic anemia is immunosuppression, since the pathophysiology of autoimmune hemolytic anemia involves immunological activation which leads to destruction of red blood cells. Suppression of the immunological activation via medications has been the cornerstone of therapy for many decades.
- Corticosteroids: Corticosteroids is the major class of medications used for treatment. Corticosteroids are the first-line therapy. Efficacy of corticosteroids is approximately 70-85%.[1] The initial dose of prednisone is 1 to 1.5mg/kg orally once daily. After a response is seen, steroids should be tapered over 6-12 months. Rapid taper of steroids can result in adrenal insufficiency, which can manifest as hypotension and fatigue and can be fatal.[1] Of note, steroids are effective only for warm-antibody type autoimmune hemolytic anemia. Steroids are not effective for cold-antibody type autoimmune hemolytic anemia. Given the multiple adverse effects of steroids, it is not ideal for a patient to remain on steroids for long-term management. If long-term immunosuppression is required for control of autoimmune hemolytic anemia, and alternative immunosuppression should be attempted.
- Adverse effects: The adverse effects of corticocsteroids include immunosuppression, opportunisitic infection, bone density loss, loss of muscle mass, increased adipose deposition, hypertension, cataracts, glaucoma.
- Splenectomy: Splenectomy, or removal of the spleen, is a second-line option for autoimmune hemolytic anemia. Splenectomy is frequently considered for patients who have steroid-refractory or relapsed disease. The response rate for splenectomy is typically 66%.[1] The ideal candidate for splenectomy is one who has adequate functional status and cardiopulmonary reserve to undergo surgery. The decision to proceed with splenectomy is typically made jointly between the patient and physician, as surgical intervention carries inherent risks. Given the infectious risk for splenectomy, patients should undergo vaccination for Hemophilus influenzae, Neisseria meningitides, and Streptococcus pneumoniae. The three organisms are encapsulated bacteria which are normally eliminated by the spleen via complement-mediated opsonization.
- Adverse effects: The adverse effects of splenectomy include the inherent surgical risk, bleeding, post-operative thrombosis, post-operative pain, systemic infection (asplenic sepsis) with encapsulated organisms, and reactive thrombocytosis.
References
- ↑ 1.0 1.1 1.2 Zanella A, Barcellini W (2014). "Treatment of autoimmune hemolytic anemias". Haematologica. 99 (10): 1547–54. doi:10.3324/haematol.2014.114561. PMC 4181250. PMID 25271314.