From the Guidelines
Treatment for autoimmune hemolytic anemia (AIHA) should start with corticosteroids, such as prednisone at 1-2 mg/kg/day orally for 3-4 weeks, followed by a slow taper over several months, as this approach has been shown to be effective in managing the condition 1. The goal of treatment is to reduce the immune system's attack on red blood cells, thereby decreasing hemolysis and improving anemia.
- First-line therapy:
- Corticosteroids, such as prednisone, are the initial treatment of choice for AIHA.
- The typical dose is 1-2 mg/kg/day orally for 3-4 weeks, followed by a slow taper over several months.
- Second-line therapy:
- Rituximab (375 mg/m² weekly for 4 weeks) may be considered for patients who do not respond adequately to steroids or require steroid-sparing options.
- Other immunosuppressive agents like azathioprine (2-3 mg/kg/day), cyclophosphamide, or mycophenolate mofetil can be used in steroid-resistant cases.
- Supportive care:
- Folic acid supplementation (1 mg daily) is essential to support erythropoiesis.
- Blood transfusions may be necessary for severe anemia, but require careful cross-matching due to autoantibodies. It is essential to monitor patients regularly for treatment response, medication side effects, and potential complications, such as infections and metabolic disorders associated with long-term steroid use 1.
From the Research
Treatment Approaches for Autoimmune Hemolytic Anemia
- The treatment for autoimmune hemolytic anemia (AIHA) is largely based on small prospective trials, case series, and empirical observations 2.
- AIHA can be divided into warm and cold types, and treatment approaches vary depending on the type and presence of an underlying disorder 2, 3.
Warm Antibody Type AIHA Treatment
- First-line treatment for warm antibody type AIHA is corticosteroids, to which most patients respond, although relapse can occur and maintenance doses are frequently required 2, 3, 4.
- Splenectomy is an effective second-line treatment and can provide long-term remission without medication 2, 3, 4.
- Rituximab is a useful alternative for steroid-refractory patients, those requiring high maintenance doses, and unfavorable candidates for surgery 2, 3, 5, 6.
Cold Agglutinin Disease Treatment
- Primary cold agglutinin disease has been recognized as having a lymphoproliferative monoclonal origin and is unresponsive to both steroids and splenectomy 2.
- Rituximab is currently the best therapeutic alternative for cold agglutinin disease, and several treatment regimens are available with variable responses 2, 3, 6.