Recurrent nonbacterial thrombotic endocarditis: A novel therapeutic approach

We present the case of a 29-year-old male with a history of Mizuho hemolytic anemia, a rare form of unstable hemoglobinopathy, who presented with congestive heart failure secondary to recurrent valve thrombosis despite appropriate oral anticoagulation. He subsequently required mitral and aortic valve replacement. Pathologic examination revealed extensive nonbacterial thrombotic endocarditis. Due to recurrent thrombosis despite therapeutic anticoagulation, we elected to treat him with red blood cell exchange transfusions and hydroxyurea. He has remained free of symptoms for almost two years with this treatment regimen without side effects. <Learning objective: The management of nonbacterial thrombotic endocarditis (NBTE) is well described in the literature. However, the treatment for refractory NBTE in aggressive forms of unstable hemoglobinopathy remains difficult. The use of hydroxyurea and serial red blood cell exchange, in addition to the usual therapies, may be useful in the treatment of refractory NBTE.>.