Un caso insolito di trombocitopenia

Abstract

A two-and-a-half-year-old child presents with spontaneous ecchymosis. In addition to severe thrombocytopoenia, initial investigations show the presence of neutropoenia and signs of immune-mediated haemolysis (positivity of the direct antiglobulin test). This raises the suspicion of Evans syndrome, since this syndrome is characterized, according to the current definition, by the destruction of at least two medullary cell lines in the absence of other diagnoses. Evans syndrome in many cases can be secondary to various pathological conditions. Investigations are carried out in the infectious, immunological, rheumatological and genetic fields, and lead to the identification of a mutation that is one of the causes of common variable immunodeficiency (CVID). So, on that ground, it can be affirmed that the present case of Evans syndrome is secondary to CVID.