Treatment of Evans Syndrome
The first-line treatment for Evans Syndrome should be corticosteroids (prednisone 1-2 mg/kg/day) with or without intravenous immunoglobulin (IVIG) for cases that require a rapid response. 1
Diagnostic Evaluation
Before initiating treatment, a comprehensive diagnostic workup should include:
- Complete blood count with differential
- Peripheral blood smear
- Reticulocyte count
- Direct Coombs test (DAT)
- LDH and haptoglobin
- Bilirubin (direct and indirect) levels 1
Additional studies may be necessary:
- Bone marrow evaluation
- Tests to detect underlying diseases
- CT scan to evaluate lymphadenopathy or splenomegaly 1, 2
Treatment Algorithm
First-Line Treatment
Treatment should be based on hemolysis grade:
| Grade | Treatment |
|---|---|
| 1-2 | Prednisone 0.5-1 mg/kg/day |
| 3 | Prednisone 1-2 mg/kg/day and IVIG 0.4-1 g/kg/day for 3-5 days if rapid response is needed |
| 4 | Methylprednisolone 1 g IV daily for 3 days, therapeutic plasma exchange, and rituximab in refractory cases [1] |
The duration of corticosteroid treatment should be 2-4 weeks, followed by a gradual reduction over 4-6 weeks to the lowest effective dose 1.
Second-Line Treatment
For patients who fail to respond to first-line therapy or relapse:
Rituximab is strongly recommended as:
Thrombopoietin receptor agonists for chronic ITP component, especially with history of severe infections 1, 2
Immunosuppressive agents such as:
- Cyclosporine
- Mycophenolate mofetil
- Azathioprine 3
Third-Line and Beyond
Splenectomy for patients who do not respond to corticosteroids, IVIG, or rituximab 1, though responses are often less durable than in uncomplicated ITP 3
Fostamatinib as third-line or further treatment, particularly for patients with previous thrombotic events 2
Stem cell transplantation for very severe and refractory cases, offering the only chance of long-term cure 3
Supportive Care
- Erythropoietin with or without IV iron may be useful as supportive therapy 1
- Avoid unnecessary transfusions as they can worsen hemolysis 1
- When transfusions are necessary, use extended compatibility matching and consult with blood bank regarding appropriate products 1
Monitoring and Follow-Up
Regular monitoring is essential:
- Hemoglobin
- Platelets
- Reticulocytes
- LDH
- Haptoglobin 1
Prognosis and Complications
Evans syndrome has a chronic and often relapsing course with significant mortality. Only about one-third of patients achieve remission off treatment 1.
Common complications include:
Factors that negatively affect survival include:
- Advanced age
- Severe anemia at onset
- Relapses
- Infections
- Thrombosis 1
Special Considerations
- Rituximab should be avoided in patients with immunodeficiency or severe infections 2
- Splenectomy should be avoided in patients with immunodeficiency or severe infections 2
- For Evans syndrome secondary to lymphoproliferative disorders, consider combination of rituximab plus bendamustine 2
Early consultation with a hematologist is recommended for moderate to severe cases to guide appropriate management 1.