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Algorithm for managing warm autoimmune hemolytic anemia (AIHA)

Algorithm for managing warm autoimmune hemolytic anemia (AIHA)
Refer to UpToDate for additional information on the evaluation, diagnosis, and management of warm AIHA and the appropriate treatments for various underlying conditions.
AIHA: autoimmune hemolytic anemia; DVT: deep vein thrombosis; PE: pulmonary embolism; Ig: immunoglobulin; RBC: red blood cell; CNS: central nervous system.
* Hemolytic anemia with positive direct antiglobulin test (DAT) for IgG and/or C3d and absence of cold-induced findings (Raynaud phenomenon, RBC agglutination in the collection tube or on the blood smear).
¶ Concerning symptoms of severe anemia include hemodynamic compromise, dyspnea, or symptoms suggestive of CNS or cardiac ischemia.
Δ We suggest glucocorticoids plus rituximab rather than glucocorticoids alone in most patients as long as there is not a contraindication. Reasons to avoid rituximab include concerns about high risk of toxicity, risk for hepatitis B reactivation, lack of access to the drug, excessive burdens of therapy, and/or mild hemolysis for which the benefit/risk calculation is unfavorable.
  • A typical initial glucocorticoid dose of prednisone is 1 to 2 mg/kg orally per day.
  • A typical rituximab dose is 375 mg/m2 intravenously once per week for 4 weeks.
Options include cyclophosphamide, mycophenolate mofetil, azathioprine, danazol, cyclosporin, sirolimus, and others. The choice among them is individualized.
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