Evans syndrome in infants

Abstract

Background

Evans’ syndrome is characterized by the reduction of at least two blood cell lines in the absence of other diagnoses; it was previously described as the simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia with unknown etiology. Incidence of 37% and mortality rate of 10% have been reported for Evans’ syndrome.

Clinical cases

We report the clinical presentation and evolution of Evans’ syndrome in two infants who were initially diagnosed with immune thrombocytopenia. The clinical diagnosis was supported by complementary studies and hematological disorders were corroborated. Both cases received treatment with steroids and intravenous immunoglobulin.

Conclusions

Other cell line disorders must be looked for when approaching children with thrombocytopenia. In the present cases, we found autoimmune hemolytic anemia and monocytosis. Therefore, infectious and immunological studies must be included. The treatment of choice are steroids, and intravenous immunoglobulin should be considered if severe immune thrombocytopenia is associated, as observed in these cases.