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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Medical Therapy

Medical treatment of autoimmune hemolytic anemia is summarized below:

Medication	Mechanism of action	Response rate	Dosing and Administration	Adverse effects	Notable features
Corticosteroids	Inhibition of IL-2 Inhibition of arachidonic acid production Inhibition of NF-kappaB signaling	70-85% Response usually occurs within 2 weeks	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	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
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
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
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
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
Cyclophosphamide	DNA alkylating agent Inhibits T cell proliferation	Variable	50 mg/kg daily for 4 days	Bone marrow suppression, nausea, vomiting, hemorrhagic cystitis, bladder cancer	Chemotherapeutic agent

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%. 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. 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.

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