Thrombotic Complications in Patients with Immune-Mediated Hemolysis

Autoimmune hemolytic anemias are rare and heterogeneous disorders characterized by hemolysis, which is a well-recognized risk factor for thrombosis. The most common immune-mediated anemias are represented by autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria, both associated with a high rate of thrombosis. Multiple pathophysiological mechanisms for thrombosis have been proposed, involving hemolysis itself and additional effects of the immune system. Despite the increasing awareness of the thrombotic risk in these conditions, evidence-based guidance on prevention and management of thrombotic events is lacking. We herein report available evidence on epidemiological data on thrombosis and thrombophilia in immune-mediated hemolysis, together with possible underlying pathophysiological mechanisms. In addition, we summarize current recommendations for treatment of thrombosis in immune-mediated hemolysis. In particular, we address the issue of thrombotic complications treatment and prophylaxis by proposing a therapeutic algorithm, focusing on specific situations such as splenectomy and pregnancy.

Automated summary

Autoimmune hemolytic anemias, including autoimmune hemolytic anemia and other immune-mediated anemias, are often associated with a high rate of thrombosis, but lack evidence-based guidance on prevention and management of thrombotic events. Pathophysiological mechanisms for thrombosis in these conditions may involve hemolysis itself and other effects of the immune system. Specific situations, like splenectomy and pregnancy, require particular attention for treatment and prevention of thrombotic complications.