Autoimmune hemolytic anemia medical therapy

Autoimmune hemolytic anemia Microchapters
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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	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	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	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	Hepatic metabolism to 6-mercaptopurine and 6-thiouric acid	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	Unknown	Higher cost of therapy than corticosteroids
Mycophenolate mofetil	Noncompetitive, selective, reversible inhibitor of inosine monophosphate (IMP) dehydrogenase Inhibits T cell proliferation by inhibiting purine synthesis	Variable	1-1.5 g PO every 12 hours	Hyperglycemia Hyperlipidemia Leukopenia Infections	Enterohepatic recirculation to MPA, the active form of mycophenolate mofetil	Higher cost of therapy than corticosteroids
Cyclosporine A	Inhibits calcineurin-mediated NFAT dephosphorylation and activation (calcineurin inhibitor) Inhibits T cell proliferation	Variable	1 mg/kg PO every 12 hours	Tremor Nephrotoxicity Hypertension Infection Headache Gingival hyperplasia	VIa hepatic CYP3A4 to metabolites AM1, AM9, and AM4N	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 and vomiting Hemorrhagic cystitis Bladder cancer	Hepatically metabolized to 4-hydroperoxycyclophosphamide and 4-aldophosphamide	Chemotherapeutic agent