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], Irfan Dotani [3]

Overview

The mainstay of therapy for autoimmune hemolytic anemia is immunosuppression, since the pathophysiology of autoimmune hemolytic anemia involves immunological activation which leads to the destruction of red blood cells. Suppression of the immunological activation via medications has been the cornerstone of therapy for many decades. Medications include corticosteroids, azathioprine, rituximab, mycophenolate mofetil, cyclosporine A, and cyclophosphamide.

Medical Therapy

Medical treatment of autoimmune hemolytic anemia is summarized below:

Medication Mechanism of action Response rate Dosing and Administration Adverse effects Metabolism Notable features

Corticosteroids

  • 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]
  • Extensive hepatic metabolism
  • First-line therapy is 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[2]

  • 40-60%
  • 1-3 mg/m2 IV weekly for 4 weeks

Rituximab[3]

  • 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
  • Unknown

Mycophenolate mofetil

  • Variable
  • 1-1.5 g PO every 12 hours

Cyclosporine A

  • Variable
  • 1 mg/kg PO every 12 hours
  • VIa hepatic CYP3A4 to metabolites AM1, AM9, and AM4N

Cyclophosphamide[2]

  • Variable
  • 50 mg/kg daily for 4 days
  • Hepatically metabolized to 4-hydroperoxycyclophosphamide and 4-aldophosphamide

References

  1. 1.0 1.1 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.
  2. 2.0 2.1 Salama A (2015). "Treatment Options for Primary Autoimmune Hemolytic Anemia: A Short Comprehensive Review". Transfus Med Hemother. 42 (5): 294–301. doi:10.1159/000438731. PMC 4678315. PMID 26696797.
  3. Fozza C, Longinotti M (2011). "Use of rituximab in autoimmune hemolytic anemia associated with non-hodgkin lymphomas". Adv Hematol. 2011: 960137. doi:10.1155/2011/960137. PMC 3087411. PMID 21547266.

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