Evans syndrome

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ICD9 = | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = ped | eMedicineTopic = 721 | MeshID = 'Evans Syndrome' is an autoimmune disease in which an individuals antibodies attack their own RBCs as well as their platelets, both of these events may occur simultaneously or one follow on from the other. Its overall pathology approximates to a combination of the two autoimmune induced conditions: autoimmune hemolytic anemia and immune thrombocytopenic purpura.Autoimmune hemolytic anemia is a condition in which the red blood cells that normally carry oxygen and carbon dioxide are destroyed by an autoimmune process. Immune thrombocytopenic purpura is a condition in which the platelets in the blood are destroyed by an autoimmune process. Platelets are a component of blood that contribute to the formation of blood clots in the body to prevent bleeding.

Epidemiology

Signs and symptoms

It has been variously reported that between 10% and 23% of patients who have autoimmune haemolytic anaemia, will also have thrombocytopenia and thus Evans syndrome. The two features may occur together or sequentially.

Causes

The precise cause is not yet known, but the immunology does differ from patients with just ITP with decreased T4 (T-helper), increased T8 (T-suppressor) and a decreased T4:T8 ratio. Together with decreased serum levels of IgG, IgM, and IgA these findings suggest an unsuccessful autoimmune response to an unknown trigger.

Diagnosis

The diagnosis is made upon blood tests to confirm not only haemolytic anaemia and immune thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying aetiology.Other antibodies may occur directed against neutrophils and lymphocytes, and "immunopancytopenia" has been suggested as a better term for this syndrome.

Treatment

Initial treatment is with glucocorticoid corticosteroids or intravenous immunoglobulin, and particularly in children autoimmune hemolytic anemia is often an acute illness that responds in 80% to a short steroid course.Although the majority of cases initially respond well, the condition often relapses and immunosuppressive drugs (e.g. ciclosporin, mycophenolate mofetil, vincristine and danazol) are then used, or combinations of these.A drug, Rituxan, has given some good results in acute and refractory Evans syndrome cases, although further relapse may occur within a year.Surgical splenectomy is also used in some cases, but may give only transient benefit in some.The only prospect for a permanent cure is the high-risk option of an allogeneic hematopoietic stem cell transplantation (SCT).

Prognosis

Evan's Syndrome is rare, serious, and has a reported mortality rate of 7%. It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia, with recent research finding that 58% of children with Evans syndrome have CD4-/CD8- T cells which is a strong predictor for having autoimmune lymphoproliferative syndrome.

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