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What is Evans syndrome?

Evans syndrome is an autoimmune disorder in which antibodies attack red blood cells (RBC), which deliver oxygen to body tissues, platelets (which help blood clot), and/or neutrophils (a type of white blood cell). The disorder resembles a combination of自身免疫性溶血性贫血(the destruction of young red blood cells), thrombocytopenia (too few platelets), andneutropenia(中性粒细胞太少)。这些可能同时发生,或者一个可能跟随另一个。

There is no known genetic cause of Evans syndrome, and occurrences in families are rare. It is considered a chronic disease, with periods of remission and exacerbation. Thrombocytopenia recurs in about 60 percent of patients, while autoimmune hemolytic anemia (AIHA) recurs in about 30 percent.

What are the symptoms of Evans syndrome?

The symptoms of Evans syndrome may be similar in nature toleukemiaand lymphoma; these illnesses must be ruled out before a diagnosis is made.

如果孩子的红细胞计数低(RBC),则可能包括:

If a child has a low platelet count, symptoms may include:

  • increased bruising
  • petechia: tiny red dots under the skin that are a result of very small bleeds into the skin
  • increased bleeding symptoms, such as abloody noseor heavy menses

If a child has a low neutrophil count, symptoms may include:

  • fevers
  • mouth sores
  • increased bacterial infections

What causes Evans syndrome?

The current cause for Evans syndrome is unknown.

How we care for Evans syndrome

Children with Evans syndrome are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. OurBlood Disorders Center为包括埃文斯综合征在内的多种非恶性血液疾病提供全面的诊断,治疗和预防服务。您的孩子会从世界上一些最有经验的人那里得到照顾血液学家.

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。

Evans Syndrome |Programs & Services

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