Multifactorial thrombotic risk in Evans syndrome: a case report and review of the literature.

Abstract

BACKGROUND: Evans syndrome (ES) is a rare autoimmune disorder characterized by the coexistence of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Beyond bleeding and infections complications, ES is increasingly recognized as a prothrombotic condition, particularly in the presence of multiple risk factors such as hemolysis, thrombocytopenia-related immune dysregulation, splenectomy, and exposure to thrombopoietin receptor agonists. However, the importance of antiphospholipid antibodies is less well established in ES patients. CASE PRESENTATION: We report the case of a 53-year-old male with a long-standing history of AIHA, later complicated by ITP and diagnosed as ES, who presented with acute deep vein thrombosis of the right lower extremity despite severe thrombocytopenia. His medical history was notable for prior splenectomy, recurrent venous thromboembolic events, cerebral venous sinus thrombosis, and recent exposure to eltrombopag. Laboratory evaluation revealed evidence of compensated hemolysis and persistent lupus anticoagulant positivity, while other antiphospholipid antibodies were negative. Due to profound thrombocytopenia, anticoagulation was initiated following platelet count recovery achieved with intravenous immunoglobulin and corticosteroids. Platelet counts were subsequently maintained with eltrombopag, allowing continuation of therapeutic anticoagulation without bleeding complications. CONCLUSIONS: This case highlights the complex interplay between thrombosis and thrombocytopenia in ES. Rather than a single etiologic factor, thrombotic events in ES appear to result from the accumulation of multiple prothrombotic conditions, including immune cytopenias, prior splenectomy, and treatment-related factors, with antiphospholipid antibodies potentially acting as an additional risk amplifier. Recognition of this multifactorial risk profile is essential for tailored management and prevention of thrombotic complications in patients with ES.