Santiago MC, Espinoza HS

Language: Spanish
References: 8
Page: 22-26
PDF size: 93.96 Kb.

ABSTRACT

Evans syndrome is a condition that usually affects primarily the pediatric population, described by Robert Evans in 1951. It is an autoimmune clinical entity manifested by the presence of thrombocytopenia and hemolytic anemia, which can vary in time of onset, course, or its duration; its associated with lymphoproliferatives syndromes, primary immunodeficiencies and systemic erythematous lupus. Etiology is caused by antibodies directed against platelet and red blood cells destroy such cells. Some patients with Evans syndrome have nodal lymphoid hyperactivity, decreased immunoglobulin IgA, M and G and cytopenias associated with abnormalities of T cells. The diagnosis is made with serum platelets below 150,000 per cubic millimeter and a positive Coombs test (which shows hemolytic anemia). Spontaneous remission or exacerbation are common. Some patients may have neutropenia, which exacerbates the syndrome. It is a chronic, recurrent and potentially fatal. The main treatment of the Evans syndrome is the corticosteroids, nevertheless the intravenous immuno-globuline, splenectomy, azatioprine and the cyclosporin are the other options of treatment.

REFERENCES

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