Evans Syndrome |诊断和治疗
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 ofanemia
- 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,对于严重的,难以治疗的案例,也是长期治愈的最佳机会
What is the long-term outlook for children with Evans syndrome?
对于埃文斯综合征的儿童,血小板减少症,贫血和中性粒细胞减少症的复发是常见的。有时可以对长期免疫抑制剂疗法保持良好的控制,并且有时患者可以自发地完全解决该疾病。