Caplacizumab in the successful management of cardiac involvement in thrombotic thrombocytopenic purpura

Abstract

Cardiac involvement is well documented in thrombotic thrombocytopenic purpura (TTP). Management remains challenging due to thrombocytopenia. Caplacizumab is a novel medication in TTP, but questions remain on its overall benefit in TTP patients. We report a 76-year-old woman who was admitted for non–ST segment elevation myocardial infarction, left systolic ventricular dysfunction, severe hemolytic anemia, and thrombocytopenia suggestive of TTP (PLASMIC score 7). Therapeutic plasma exchange (TPE) and caplacizumab were started alongside an immunosuppressive regimen. After 3 days of treatment, repeat echocardiography showed complete resolution of left ventricular dysfunction. We were able to stop TPE and start aspirin on the fourth day after normalization of platelet count. Our report outlines the potential benefits of caplacizumab for the time-sensitive management of acute coronary syndrome and the compromised volume status of heart failure patients, with early platelet recovery and lower duration of TPE.

Keywords:

• caplacizumab
• cardiomyopathy
• case report
• NSTEMI
• thrombotic thrombocytopenic purpura