Primary Evan Syndrome With Disseminated Intravascular Coagulation Suggests Progressive Immune Dysregulation and Early Immunosuppressive Intervention is Key to Improving Outcomes.

Evan syndrome (ES) is a rare hematological disorder that involves 2 or more immune cytopenias. It usually includes autoimmune hemolytic anemia and autoimmune thrombocytopenia. Although occasionally associated with immune neutropenia, its association with disseminated intravascular coagulation (DIC) is rare. And, early diagnosis with appropriate intervention is important because mortality from ES is known to be greater than that of isolated immune hemolytic anemia and probably worse in the presence of DIC. Considering that the presence of DIC can make the diagnosis of ES challenging, a strong clinical suspicion is important as early initiation of therapy is critical to reducing the morbidity and mortality associated with this syndrome. We report a case of ES complicated by DIC.