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

Davood Maleki

doi:10.4236/crcm.2014.36072

Abstract

Recurrent priapism is a rare, serious and difficult to treat complication of some hematological disorders, for which no standard therapy exists. This study reports a case of a 42-year-old man with thalassemia intermedia complicated by recurrent episodes of priapism. To prevent priapism recurrences, a trial of PDE5is use was initiated. One day after initiation of a PDE5i (25 mg sildenafil repeated every 8 hours), priapism was improved. For 3 weeks, the patient reported improvement, without experiencing any episodes of priapism and a normal physiologic erectile function. Four weeks after treatment he experienced priapism reoccurrence and doubling of the Sildenafil was not effective. Gonadotropin-releasing hormone agonist initiated and one week after initiatin of new drug he improved. He was free of priapism episodes for more than 2 years afterward. PDE5 deregulation seems to be an underling pathologic mechanism of recurrent priapism at least in thalassemia intermedia patients. It appears that PDE5is may have a role in the management of such patients and further testing in clinical trials is needed.

Highlights

Priapism is an abnormal persistent erection of the penis
A few case reports of recurrent priapism occurring in thalassemia intermedia especially after splenectomy [6] [7], hemoglobin-E beta thalassemia [8], hereditary spherocytosis [9] and glucose6-phosphate dehydrogenase (G6PD) deficiency [10] [11] have been documented in the literature
Episodes of recurrent priapism are treated by home intracavernous self-injections of the alpha-adrenergic agonists [19] [20]

Summary

Introduction

Priapism is an abnormal persistent erection of the penis. Stuttering or recurrent priapism is characterised by spontaneous and prolonged erections, lasting up to 3 hours, How to cite this paper: Maleki, D. (2014) Oral Phosphodiesterase Type 5 Inhibitors in Recurrent Priapism Complicating Thalassemia Intermedia: A Case Report. A few case reports of recurrent priapism occurring in thalassemia intermedia especially after splenectomy [6] [7], hemoglobin-E beta thalassemia [8], hereditary spherocytosis [9] and glucose6-phosphate dehydrogenase (G6PD) deficiency [10] [11] have been documented in the literature. Recurrent priapism is a rare but serious and difficult to treat condition. The pathophysiology of this type of priapism is not fully elucidated [12] [13]. No standard therapy exists for the prevention of recurrent ischemic priapism [17] [18]

Case Report

Discussion