Hemophagocytic Syndrome: Report of 4 cases in Hospital Maria, Especialidades Pediátricas

Abstract

Background: Hemophagocytic Syndrome (HS) is caused by an excessive or inadequate response of the immune system to a trigger or genetic deficiencies, which results in an exaggerated inflammatory reaction. It can be classified as primary if associated with hereditary factors or primary immunodeficiency's, or secondary, associated with infections, oncology or autoimmune diseases.

Description of clinical cases: in 2016, four cases regarding Hemophagocytic Syndrome were diagnosed at Hospital Maria, Especialidades Pediátricas. Three of them secondary to infections and one due to a oncology disease. Treatment of the four patients was based on the HLH-2004 protocol.

Conclusions: the objective is to make a timely diagnosis, start opportune treatment and thus reduce mortality percentage conferred by the Hemophagocytic Syndrome.