[Evans syndrome: be careful of over-diagnosis]

Autor: H, Eddou, C, Helissey, J, Konopacki, B, Souleau, T, de Revel, J V, Malfuson
Jazyk: francouzština
Rok vydání: 2011
Předmět:
Zdroj: La Revue de medecine interne. 33(3)
ISSN: 1768-3122
Popis: Evans syndrome (ES) is characterized by the coexistence of an autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). Despite being frequently evocated in the simultaneous presence of anemia and thrombocytopenia, this rare disease only accounts for 0.8 to 3.7% of patients with ITP or AIHA.We report three suspected cases of ES, diagnosed in the presence of thrombocytopenia and hemolytic anemia association, with a positive direct Coombs test in two patients. Standard ES treatment failure and occurrence of additional features subsequently led to correct diagnosis to thrombotic thrombocytopenic purpura, myelodysplastic syndrome with AIHA, and ITP with hemorrhagic anemia, respectively.Bicytopenias, even in an immunological context, are not sufficient to ascertain ES diagnosis. Our cases illustrate the diagnostic difficulties that may arise in daily practice, and induce over-diagnosis of this rare disease.
Databáze: OpenAIRE