Síndrome de Evans na infância e perspectivas terapêuticas: revisão narrativa

Abstract

GUEDES, M.L.B. Evans syndrome in childhood and therapeutic perspectives: narrative review. 2022. Trabalho de Conclusão de Curso em Medicina - Centro de Ciências Médicas, Universidade Federal da Paraíba, João Pessoa, 2022. Introduction: Evans syndrome (ES) is a rare autoimmune disease defined by Robert Evans in 1951 when he studied the relationship between autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) in the absence of a known underlying etiology. Evans syndrome has a typical course and is characterized by being a chronic disease of heterogeneous expression, presenting clinical variability at onset, periods of remission and spontaneous exacerbations. Objectives: This work aims to analyze the therapeutic perspectives of Evans syndrome in childhood, through a review of the literature. Material and methods: this work was prepared from a narrative review of the literature, through research in bibliographic sources of lilacs, cochrane and medline databases, using as descriptors “Evans syndrome” and “child” and “therapeutic ”. 66 articles were identified and, after reading the titles, 7 articles were selected after reading the abstracts that fulfilled the initially proposed criteria and they were read in full. Results and conclusion: most of the selected articles were cohort studies, and validated the use of Rituximab, mycophenolate mofetil and sirulimus, among other therapies, as safe options for second line treatment in children and adolescents, often functioning as steroid-sparing agents.