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%	Prednisone 1 to 1.5mg/kg PO daily for 3 weeks; rapid taper down to 20mg PO daily; slow taper from 20mg to 0mg	immunosuppression, opportunisitic infection, bone density loss, loss of muscle mass, increased adipose deposition, hypertension, cataracts, glaucoma	First-line therapy
Azathioprine	Purine synthesis inhibitor Converts to 6-mercaptopurine Antibody-dependent cell-mediated cytotoxicity	Variable	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	Variable	375 mg/m2 IV weekly for 4 weeks	Hepatitis B reactivation, progressive multifocal leukoencephalopathy	Higher cost of therapy than corticosteroids
Mixed warm-antibody and cold-antibody type	Due to IgM subtype Mixed features

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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