A Case of Evans Syndrome: A Clinical Condition With Under-Recognized Thrombotic Risk

Abstract

Evans syndrome (ES) is a rare hematologic disorder characterized by the presence of Direct Antiglobulin test (DAT) positive autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and/or immune neutropenia. The risk of thrombosis has been well established in retrospective studies for ITP and AIHA; however, the risk of thrombosis in ES has been limited to case reports and individual case series. Whether the risk of thrombosis in ITP and AIHA is additive has not been well established, but preliminary observation suggests the rate of thrombosis is higher than the rate observed in ITP or AIHA individually. Furthermore, ES appears to be an underappreciated diagnosis. Anemia in the presence of ITP is often considered to be secondary to acute blood loss and a proper workup for hemolysis is often missed. Appropriate risk stratification of these patients is hindered by this lack of workup and many patients are unfortunately not treated appropriately and/or not offered appropriate prophylaxis for their level of thrombotic risk. The purpose of this case report is to not only increase the level of awareness among clinicians to initiate an appropriate diagnostic workup in patients presenting with anemia in the setting of ITP, but also to a heightened thrombotic risk warranting an appropriate thromboprophylaxis. This case also adds to the body of evidence that a “second- hit” phenomenon is often a precipitating cause for a thrombotic event.




J Hematol. 2015;4(3):205-209
doi: http://dx.doi.org/10.14740/jh222w