In france, a collaborative pediatric network set up via the national rare disease plan now provides comprehensive clinical data in children with this disease. Evans syndrome, autoimmune cytopenias, lowdose rituximab, ivig, systemic. Although few statistical data are available, it has been reported that between and 73% of patients with autoimmune hemolytic. Evans syndrome es is defined by the decrease in at least two cell lines in the absence of other diagnoses 1. Evans syndrome es is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune. Evans syndrome es, which was first described in 1951, is an autoimmune disorder characterized by the simultaneous or sequential development of autoimmune hemolytic anemia aiha and immune thrombocytopenia itp andor immune neutropenia in the absence of any underlying cause. Evans syndrome, thrombocytopenia, autoimmune hemolytic anemia, pregnancy. Evans syndrome es is an uncommon autoimmune disease that was defined by.
Evans syndrome is the coexistence of simultaneous or sequential direct coombspositive autoimmune hemolytic anemia aiha with immunemediated thrombocytopenia. In 27% to 50% of cases there is an associated malignancy or a predisposing autoimmune disease e. E uma doenca cronica e recidivante associada a morbidade significativa, apesar da terapeutica. Earlyonset evans syndrome, immunodeficiency and premature immunosenescence associated with tripeptidylpeptidase ii deficiency. In the initial description by evans et al in 1951, the anemia and thrombocytopenia varied with respect to. Management of evans syndrome university of wisconsin.
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