What is the recommended treatment for Autoimmune Hemolytic Anemia (AIHA)?

Treatment of Autoimmune Hemolytic Anemia (AIHA)

Corticosteroids are the first-line treatment for autoimmune hemolytic anemia, with prednisone or prednisolone at 1 mg/kg/day orally for 4-6 weeks, followed by a slow taper over 6-12 months. 1

Initial Treatment Based on AIHA Subtype

Warm AIHA (Most Common Type)

• First-line treatment: Prednisone/prednisolone 1 mg/kg/day PO for 4-6 weeks 1
  • Expected response rate: 70-85% of patients
  • Clinical improvement should be seen within 2 weeks
  • Slow taper over 6-12 months after response to minimize relapse risk
• Monitoring parameters: Hemoglobin, reticulocytes, LDH, and bilirubin levels 1

Cold Agglutinin Disease

• First-line treatment: Rituximab (375 mg/m² weekly for 4 weeks) 1, 2
• Supportive care: Patients must avoid cold exposure 1

Paroxysmal Cold Hemoglobinuria

• Aggressive supportive therapy with corticosteroids 3
• Hemolysis typically terminates spontaneously

Second-Line Treatment for Refractory Warm AIHA

1. Rituximab: 375 mg/m² weekly for 4 weeks 1, 2

   • Effective in 70-80% of cases
   • Now preferred over splenectomy as second-line treatment

2. Splenectomy: Alternative second-line option 1, 2

   • Effective in approximately 70% of cases
   • Potential for complete and long-term remission (cure rate up to 20%)
   • Major risk: overwhelming post-splenectomy infection

Third-Line Treatment Options

For patients failing both corticosteroids and rituximab/splenectomy:

• Immunosuppressive drugs: 1, 2
  • Azathioprine
  • Cyclophosphamide
  • Cyclosporine
  • Mycophenolate mofetil
• Danazol 2
• Intravenous immunoglobulin (IVIG) 2

Management of Severe/Life-Threatening AIHA

• High-dose intravenous methylprednisolone 1
• Blood transfusion despite compatibility issues 1
  • "Least incompatible" units may be necessary
  • Don't withhold transfusion in life-threatening anemia

Special Considerations

Secondary AIHA

• Treat underlying condition (lymphoproliferative disorders, autoimmune diseases, infections) 1, 3
• Treatment approach for hemolysis similar to primary AIHA

Pregnancy

• IVIG is relatively safe during pregnancy 1

Common Pitfalls to Avoid

1. Tapering corticosteroids too quickly - leads to relapse 1
2. Inadequate treatment duration - minimum 6 months recommended 1
3. Failure to investigate for underlying conditions in secondary AIHA 1
4. Overlooking DAT-negative AIHA (5% of cases) - specialized testing for IgA autoantibodies, warm IgM, or low-affinity IgG should be considered 1
5. Withholding transfusion in life-threatening anemia despite compatibility issues 1

Treatment Algorithm

1. Diagnose AIHA subtype (warm, cold, mixed, paroxysmal cold hemoglobinuria)
2. Initiate appropriate first-line therapy based on subtype
3. Assess response within 2-4 weeks
4. If inadequate response:
   • Confirm diagnosis and rule out secondary causes
   • Escalate to second-line therapy (rituximab or splenectomy)
5. If still refractory: Consider third-line immunosuppressive options
6. For all patients: Provide appropriate supportive care and monitor for complications