Oral Phosphodiesterase Type 5 Inhibitors Alleviate Recurrent Priapism Complicating Thalassemia Intermedia: A Case Report

Abstract

Recurrent ischemic priapism still remains a serious and difficult to treat complication of certain hematological disorders. Elucidation of the underlying pathophysiologic mechanisms and application of new effective prophylactic treatments are needed. To present the efficacy of phosphodiesterase type 5 inhibitors (PDE5is) as a preventive measure against ischemic priapism recurrences complicating thalassemia intermedia. We report on the case of a 19-year-old Caucasian man with thalassemia intermedia complicated by recurrent episodes of priapism following therapeutic splenectomy. After failure of conventional measures to control recurrences, a trial of long-term PDE5is use was initiated. PDE5is efficacy based on clinical patient history. Within 2 months of PDE5i preventive strategy, priapism recurrences nearly resolved. At 6 months, prophylaxis was discontinued. At 12 months, the patient reported clear improvement and satisfaction, experiencing rare episodes of priapism and a physiologic erectile function. PDE5 dysregulation seems to be an underline pathogenetic mechanism of thalassemia intermedia-associated priapism. It appears that PDE5is might have a role in the clinical management of such patients and their preventive efficacy warrants further testing in clinical trials.