CRCM  Vol.3 No.6 , June 2014
Oral Phosphodiesterase Type 5 Inhibitors in Recurrent Priapism Complicating Thalassemia Intermedia: A Case Report
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.
Cite this paper: Maleki, D. (2014) Oral Phosphodiesterase Type 5 Inhibitors in Recurrent Priapism Complicating Thalassemia Intermedia: A Case Report. Case Reports in Clinical Medicine, 3, 327-330. doi: 10.4236/crcm.2014.36072.

[1]   Abu Sham’a, R.A.H., Kufri, F.H. and Yassin, I.H. (2008) Stuttering Priapism Complicating Warfarin Therapy in a Patient with Protein C Deficiency. International Journal of Laboratory Hematology, 30, 339-343.

[2]   Burnett, A.L., Bivalacqua, T.J., Champion, H.C. and Musicki, B. (2006) Feasibility of the Use of Phosphodiesterase Type 5 Inhibitors in a Pharmacologic Prevention Program for Recurrent Priapism. Journal of Sexual Medicine, 3, 1077-1084.

[3]   Virag, R., Bachir, D., Lee, K. and Galacteros, F. (1996) Preventive Treatment of Priapism in Sickle Cell Disease with Oral and Self-Administered Intracavernous Injection of Etilefrine. Urology, 47, 777-781.

[4]   Rogers, Z.R. (2005) Priapism in Sickle Cell Disease. Hematology/Oncology Clinics of North America, 19, 917-928.

[5]   Prabhakaran, K., Jacobs, B.L., Smaldone, M.C. and Franks, M.E. (2007) Stuttering Priapism Associated with Hereditary Spherocytosis. The Canadian Journal of Urology, 14, 3702-3704.

[6]   Jackson, N., Franklin, I.M. and Hughes, M.A. (1986) Recurrent Priapism Following Splenectomy for Thalassaemiaintermedia. British Journal of Surgery, 73, 678.

[7]   Macchia, P., Massei, F., Nardi, M., Favre, C., Brunori, E. and Barba, V. (1990) Thalassemia Intermedia and Recurrent Priapism Following Splenectomy. Haematologica, 75, 486-487.

[8]   Sharma, R., Prakash, R., Kamboj, S. and Jain, V. (2008) Hemoglobin-E Beta Thalassemia Presenting with Recurrent Priapism—A Rare Complication. American Journal of Hematology, 83, 257.

[9]   Prabhakaran, K., Jacobs, B.L., Smaldone, M.C. and Franks, M.E. (2007) Stuttering Priapism Associated with Hereditary Spherocytosis. The Canadian Journal of Urology, 14, 3702-3704.

[10]   Burnett, A.L. and Bivalacqua, T.J. (2008) Glucose-6-Phosphate Dehydrogenase Deficiency: An Etiology for Idiopathic Priapism? Journal of Sexual Medicine, 5, 237-240.

[11]   Finley, D.S. (2008) Glucose-6-Phosphate Dehydrogenase Deficiency Associated Stuttering Priapism: Report of a Case. Journal of Sexual Medicine, 5, 2963-2966.

[12]   Tzortzis, V., Mitrakas, L., Gravas, S., Mamoulakis, C., Meissner, A., Kyriakou, D. and Melekos, M.D. (2009) Oral Phosphodiesterase Type 5 Inhibitors Alleviate Recurrent Priapism Complicating Thalassemia Intermedia: A Case Report. Journal of Sexual Medicine, 6, 2068-2071.

[13]   Wen, J., Jiang, X., Dai, Y., Zhang, Y., Tang, Y., Sun, H., Mi, T., Kellems, R.E., Blackburn, M.R. and Xia, Y. (2010) Adenosine Deaminase Enzyme Therapy Prevents and Reverses the Heightened Cavernosal Relaxation in Priapism. Journal of Sexual Medicine, 7, 3011-3022.

[14]   Burnett, A.L. (2003) Priapism Pathophysiology: Clues to Prevention. International Journal of Impotence Research, 15, S80-S85.

[15]   Champion, H.C., Bivalacqua, T.J., Takimoto, E., Kass, D.A. and Burnett, A.L. (2005) Phosphodiesterase-5A Dysregulation in Penile Erectile Tissue Is a Mechanism of Priapism. Proceedings of the National Academy of Sciences of the USA, 102, 1661-1666.

[16]   Burnett, A.L. (2008) Molecular Pharmacotherapeutic Targeting of PDE5 for Preservation of Penile Health. Journal of Andrology, 29, 3-14.

[17]   Muneer, A, Minhas, S., Arya, M. and Ralph, D.J. (2008) Stuttering Priapism—A Review of the Therapeutic Options. International Journal of Clinical Practice, 62, 1265-1270.

[18]   Abern, M.R. and Levine, L.A. (2009) Ketoconazole and Prednisone to Prevent Recurrent Ischemic Priapism. Journal of Urology, 182, 1401-1406.

[19]   McDonald, M. and Santucci, R.A. (2004) Successful Management of Stuttering Priapism Using Home Self-Injections of the Alpha-Agonist Metaraminol. International Braz J Urol, 30, 121-122.

[20]   Ralph, D.J., Pescatori, E.S., Brindley, G.S. and Pryor, J.P. (2001) Intracavernosal Phenylephrine for Recurrent Priapism: Self-Administration by Drug Delivery Implant. Journal of Urology, 165, 1632.

[21]   Levine, L.A. and Guss, S.P. (1993) Gonadotropin-Releasing Hormone Analogues in the Treatment of Sickle Cell Anemia-Associated Priapism. Journal of Urology, 150, 475-477.

[22]   Dahm, P., Rao, D.S. and Donatucci, C.F. (2002) Antiandrogens in the Treatment of Priapism. Urology, 59, 138.

[23]   Rachid-Filho, D., Cavalcanti, A.G., Favorito, L.A., Costa, W.S. and Sampaio, F.J. (2009) Treatment of Recurrent Priapism in Sickle Cell Anemia with Finasteride: A New Approach. Urology, 74, 1054-1057.

[24]   Khot, R. and Aher, A. (2012) Sickle Cell Disease with Recurrent Priapism. The Journal of the Association of Physicians of India, 60, 62-63.

[25]   Rourke, K.F., Fischler, A.H. and Jordan, G.H. (2002) Treatment of Recurrent Idiopathic Priapism with Oral Baclofen. Journal of Urology, 168, 2552-2553.