From the Guidelines
Treatment for autoimmune hemolytic anemia should begin with corticosteroids, primarily prednisone at 1-2 mg/kg/day for 3-4 weeks, followed by a slow taper over several months, as this approach has been consistently supported by recent guidelines and studies 1. The initial treatment with corticosteroids aims to reduce the autoimmune destruction of red blood cells, and the majority of patients respond well to this therapy.
- Key points to consider in the treatment of autoimmune hemolytic anemia include:
- The use of rituximab (375 mg/m² weekly for 4 weeks) as a second-line therapy for patients who do not respond adequately to steroids 1.
- The consideration of splenectomy as an option for steroid-refractory cases, particularly in warm AIHA, although this should be weighed against the potential risks and benefits 1.
- The potential use of immunosuppressants like azathioprine (1-2 mg/kg/day), mycophenolate mofetil (500-1000 mg twice daily), or cyclosporine for maintenance therapy in selected cases 1.
- The importance of supportive care, including folic acid supplementation (1 mg daily), blood transfusions for severe anemia, and treating any underlying conditions 1. It is essential to note that the treatment approach should be individualized based on the specific characteristics of the patient and the disease, and that the goal of treatment is to improve morbidity, mortality, and quality of life.
- Recent studies have highlighted the importance of considering the underlying cause of autoimmune hemolytic anemia, such as chronic lymphocytic leukemia (CLL), and tailoring the treatment approach accordingly 1. In general, the treatment of autoimmune hemolytic anemia should prioritize the use of corticosteroids as the initial therapy, with rituximab and other options considered for patients who do not respond adequately to steroids or have specific disease characteristics that warrant alternative approaches 1.
From the Research
Treatment Options for Autoimmune Hemolytic Anemia
- The treatment of autoimmune hemolytic anemia (AIHA) is not evidence-based, but various options are available depending on the type of AIHA [(2,3,4,5)].
- For warm AIHA, corticosteroids are the first-line therapy, effective in 70-85% of patients, and should be slowly tapered over 6-12 months [(2,3,4,6)].
- Refractory or relapsed cases of warm AIHA may be treated with:
- Splenectomy, effective in approximately 70% of cases, but with a presumed cure rate of 20% [(2,3,4)].
- Rituximab, effective in approximately 70-90% of cases, and is becoming the preferred second-line treatment [(2,4,5)].
- Immunosuppressive drugs, such as azathioprine, cyclophosphamide, cyclosporin, and mycophenolate mofetil [(2,3,4)].
- For cold agglutinin disease (CAD), rituximab is now recommended as first-line treatment [(2,4,5)].
- Additional therapies for AIHA include:
- Intravenous immunoglobulins [(2,3,4)].
- Danazol [(2,3,4)].
- Plasma-exchange [(2,3,4)].
- Alemtuzumab and high-dose cyclophosphamide as last resort options [(2,4)].
- New drugs are under development, including B-cell directed therapies and inhibitors of complement, spleen tyrosine kinases, or neonatal Fc receptor 5.