Sickle cell-induced ischemic priapism

Abstract

Priapism is one of the complications of Sickle cell disease (SCD). Priapism can be classified into ischemic (low-flow), non-ischemic (high-flow), or recurrent (stuttering). Ischemic priapism affects up to half of all men with SCD. Therefore, education coupled with more research on treatment options may help the patients with SCD to avoid the social, psychological, and medical implications of this condition and its embarrassing complications. This paper will focus on the mechanisms underlying the development of ischemic priapism in SCD, current preventative treatment strategies, and future directions for improved management of this condition.

Keywords:

• ischemic priapism
• sickle cell disease (SCD)
• hemoglobin S point mutation (HbS)

Public Interest Statement

Prompt identification and proper treatment of a priapism episode in males with sickle cell disease (SCD) is serious, as the end result of prolonged episodes of priapism can be ischemia and fibrosis in the corpus cavernosa of the penis, thereby leading to impaired sexual function and impotence. The type and severity of the complications of SCD vary depending on a variety of genetic factors including the presence of other types of hemoglobin, particularly persistent fetal hemoglobin (HbF). Priapism is a real emergency, and early intervention allows the best chance of functional recovery. Any therapeutic possibility that aids comfort and decreases the possibility of surgery and thus the likelihood of erectile dysfunction would be of great benefit to patients with ischemic priapism. There research for finding new preventative treatments including long-term low-dose PDE5 inhibitors to normalize NO downstream signaling and potential inhibition of adenosine signaling to minimize fibrosis following an episode of priapism.

Competing Interests

The authors declare no competing interests.

Acknowledgment

The authors are thankful to www.manuscriptedit.com for providing English language editing and proofreading services for this manuscript.

Additional information

Notes on contributors

Salma AlDallal

Dr Salma AlDallal is currently the chairman of Laboratory Utilization Committee of the prestigious Amiri Hospital, Ministry of Health. She has completed her PhD in Cancer studies “Molecular genetics of Lymphoma” from University of Manchester, Manchester, UK. She has more than 12 years of professional experience in the field of health and medical sciences. She has been the member of research team in Haematology Department at Ministry of Health-Amiri Hospital. She has published several scholarly papers in many renowned international and national journals. She is the member of the British Society for Haematology and American Society of Haematology.