Autoimmune hemolytic anemia medical therapy
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Autoimmune hemolytic anemia Microchapters |
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Differentiating Autoimmune hemolytic anemia from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Autoimmune hemolytic anemia medical therapy On the Web |
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American Roentgen Ray Society Images of Autoimmune hemolytic anemia medical therapy |
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Directions to Hospitals Treating Autoimmune hemolytic anemia |
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Risk calculators and risk factors for Autoimmune hemolytic anemia medical therapy |
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
Medical treatment of autoimmune hemolytic anemia is summarized below:
| Medication | Mechanism of action | Response rate | Dosing and Administration | Adverse effects | Notable features |
|---|---|---|---|---|---|
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Corticosteroids |
Inhibition of IL-2 Inhibition of arachidonic acid production Inhibition of NF-kappaB signaling |
70-85% Response usually occurs within 2 weeks[1] |
Prednisone 1 to 1.5mg/kg PO daily for 1-3 weeks until hemoglobin improves to 10g/dl; rapid taper down to 20mg PO daily; slow taper over months from 20mg to 0mg Treat for 3-4 months with low-dose prednisone[1] |
immunosuppression, opportunisitic infection, bone density loss, loss of muscle mass, increased adipose deposition, hypertension, cataracts, glaucoma |
First-line therapy Co-administer calcium supplementation with vitamin D (for bone protection) Co-administer H2 receptor antagonist for GI protection if high risk for gastrointestinal bleeding |
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Azathioprine |
Purine synthesis inhibitor Converts to 6-mercaptopurine Antibody-dependent cell-mediated cytotoxicity |
40-60% |
1-3 mg/m2 IV weekly for 4 weeks |
Hepatitis B reactivation, progressive multifocal leukoencephalopathy |
Higher cost of therapy than corticosteroids |
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Rituximab |
CD20 monoclonal antibody Antibody-dependent cell-mediated cytotoxicity Depletion of B cells |
83-87% overall response rate 54-60% complete response rate |
375 mg/m2 IV weekly for 4 weeks 100 mg IV weekly for 4 weeks |
Hepatitis B reactivation, progressive multifocal leukoencephalopathy, infusion reaction |
Higher cost of therapy than corticosteroids |
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Mycophenolate mofetil |
Inosine monophosphate dehydrogenase inhibitor Inhibits T cell proliferation |
Variable |
1-1.5 g PO every 12 hours |
Hyperglycemia, hyperlipidemia, leukopenia, infections |
Higher cost of therapy than corticosteroids |
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Cyclosporine A |
Calcineurin inhibitor Inhibits T cell proliferation |
Variable |
1 mg/kg PO every 12 hours |
Tremor, nephrotoxicity, hypertension, infection, headache, gingival hyperplasia |
Nephrotoxicity limits its use in patients with renal dysfunction |
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.