Evan's Syndrome | Diagnosis & Treatment

How is Evans syndrome diagnosed?

Doctors diagnose Evans syndrome with a variety of blood tests, including:

  • Complete blood count
  • Coombs test: A blood test that identifies the cause of anemia
  • Reticulocyte count: A blood test that measures how fast new red blood cells called reticulocytes are made by bone marrow and released into the blood

Other tests may include bone marrow aspiration and biopsy and flow cytometry, a technique for identifying and sorting cells by staining with a fluorescent dye. After all tests are completed, doctors will be able to outline the best treatment options. 

What are the treatments for Evans syndrome?

The first-line treatments for Evans syndrome are corticosteroid, such as prednisone, and intravenous immunoglobulin, which help to control acute episodes. Most children respond to this treatment, but relapses frequently occur. Other treatments include:

  • immunosuppressive drugs, such as 6-mercaptopurine and mycophenolate mofetil
  • removal of the spleen to improve blood counts, which may be short-lived
  • rituximab, a monoclonal antibody
  • stem cell transplant, for severe, difficult-to-treat cases and the best chance for a long-term cure 

What is the long-term outlook for children with Evans syndrome? 

For children with Evans syndrome, recurrences of thrombocytopenia, anemia, and also neutropenia are common. It can sometimes remain well controlled on long-term immunosuppressant therapy and occasionally patients have spontaneous complete resolution of the condition.