Initial Treatment for Autoimmune Hemolytic Anemia
High-dose corticosteroids are the first-line treatment for autoimmune hemolytic anemia, with the specific regimen determined by disease severity. 1
Treatment Algorithm Based on Hemoglobin Level
The initial approach should be stratified by hemoglobin level and symptom severity:
Severe AIHA (Hemoglobin < 8.0 g/dL or Transfusion Required)
Administer prednisone 1-2 mg/kg/day (oral or IV depending on symptoms) immediately. 1 For acute, severe presentations, high-dose intravenous methylprednisolone (≥1 mg/kg) should be given as early as possible. 1 Hospital admission for close monitoring is recommended in these cases. 1
- Consider adding rituximab (375 mg/m² weekly for 4 weeks) upfront in severe cases, as early addition improves outcomes. 2, 3
- RBC transfusion should be used for symptomatic patients, using the minimum units necessary. 1
Moderate AIHA (Hemoglobin 8.0-10.0 g/dL)
Start prednisone 0.5-1 mg/kg/day orally. 1 This dose is appropriate for patients with moderate hemolysis who are not severely symptomatic.
Mild AIHA (Hemoglobin < Lower Limit of Normal to 10.0 g/dL)
Close clinical follow-up and laboratory monitoring without immediate treatment may be appropriate. 1
Monitoring Treatment Response
The treatment goal is complete normalization of hemoglobin and other laboratory parameters. 1 Monitor response through:
- Hemoglobin levels
- Reticulocyte count
- Direct antiglobulin test (DAT)
- Bilirubin, LDH, and haptoglobin 1
Continue the initial high dose of prednisone (60 mg daily or 1-2 mg/kg/day) until the hematocrit reaches 30% or hemoglobin normalizes. 4, 5 Then initiate slow, progressive dose reduction. 4
When First-Line Therapy Fails
If prednisone doses greater than 15 mg daily are required to maintain remission, this constitutes treatment failure. 4 If no response occurs after one week of prednisone, add azathioprine 2.0-2.5 mg/kg. 4
Rituximab (375 mg/m² weekly for 4 weeks) is the preferred second-line treatment with 70-80% effectiveness. 1 This should be considered early in severe cases and if no prompt response to steroids is achieved. 2
Critical Pitfalls to Avoid
Do not use IV anti-D in patients with autoimmune hemolytic anemia, as it will exacerbate hemolysis. 6 This agent is contraindicated in AIHA despite its use in immune thrombocytopenia.
Transfusion in AIHA requires careful pretransfusion workup to exclude underlying alloalloantibodies. 7 The autoantibody complicates crossmatching, but transfusion services can usually provide safe blood. 7 When transfusion is urgently required and compatible blood cannot be located, incompatible blood may be provided as a life-saving measure. 7
Third-Line Options for Refractory Disease
If both corticosteroids and rituximab fail:
- Cyclophosphamide 1-2 mg/kg/day 1
- Cyclosporine 3 mg/kg/day (adjusted for target trough levels 100-150 ng/mL) 1
- Splenectomy (has potential for complete long-term remission but carries risk of overwhelming postsplenectomy infection) 5
- Intravenous immunoglobulin (IVIG) 0.3-0.5 g/kg for rapid but temporary improvement 1