Ischemic priapism in sickle cell disease as a cause of sexual dysfunction. Case series and literature review

Priapismo isquémico en la enfermedad falciforme como causa de disfunción sexual. Serie de casos y revisión de la literatura

Published 2024-03-10

Open | Download
PDF (Spanish) FLIP
Issue
Vol. 10 No. 2 (2023): Julio - Diciembre
Section
Original articles
How to Cite
Ischemic priapism in sickle cell disease as a cause of sexual dysfunction. Case series and literature review.
Rev. colomb. hematol. oncol. [Internet]. 2024 Mar. 10 [cited 2024 Dec. 22];10(2):12-21. Disponible en: https://doi.org/10.51643/22562915.664
DOI

https://doi.org/10.51643/22562915.664
Dimensions
PlumX
license
Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Español
Universidad de Cartagena, Facultad de Medicina. Cartagena, Colombia
    https://orcid.org/0000-0001-9790-7124
    Rita Magola Sierra Merlano
    Español
      https://orcid.org/0000-0003-2775-0760
      Keyner Toro Osorio
      Español
        https://orcid.org/0000-0001-9441-5693
        Show authors biography

        Español,

        Médico. Especialista en Medicina Interna.

        Rita Magola Sierra Merlano,

        Médica. Especialista en Medicina Interna y Reumatología. PhD Ciencias de la Educación. 

        Keyner Toro Osorio,

        Médico. Especialista en Medicina Interna y Vascular.

        Sickle cell disease (SCD) comprises a group of hereditary disorders with heterogeneous clinical expression and multiple complications of a vascular and hemolytic nature. Priapism represents a urological emergency that requires early surgical intervention to reduce erectile dysfunction. Objectives: Report five cases of SCD treated for priapism at the ESE University Hospital of Cartagena (ESEHUC), and highlight the sociodemographic, clinical and laboratory characteristics that were associated with erectile dysfunction. Methods: Hospital case series. Results: Five cases of priapism were recorded with a median calculated for age of 33 years, BMI: 23 kg/m2, three cases belonged to the homozygous subtype, one double heterozygous, and one sickle cell trait. The average hospital admission was considered late, being greater than 48 hours of evolution of priapism and urological surgical management was chosen in all of them. Three cases presented sexual dysfunction, one was classified as severe. Conclusions: Cases with subclinical forms and low-risk genotypes may face greater priapism, with a risk of complications with an impact on sexual life. We insist on educating patients about: early consultation, and lifestyles that reduce the risk of priapism.

        Article visits 262 | PDF visits 231

        1. Sundd, Prithu, Gladwin, Mark, Novelli E. Pathophysiology of Sickle Cell Disease [Internet]. Annu Rev Pathol. 2019;14:263–92. Disponible en: https://doi.org/10.1146/annurev-pathmechdis-012418-012838.
        2. Ramírez-cheyne J, Moreno M, Mosquera S, Duque S, Holguín J, Camacho A. Primeros dos años de notificación de las enfermedades huérfanas-raras en Cali e identificación de algunas variables asociadas con la mortalidad. Iatreia. 2020;33(2):111–22. Disponible en: https://doi.org/10.17533/udea.iatreia.37.111
        3. Castillo, Martha, Mora, Ana, Oliveros A. Anemias ferropénicas asociadas a hemoglobinopatías en comunidades afrodescendientes en Colombia. Nova [Internet]. 2018;16(29):33–8. Disponible en: https://hemeroteca.unad.edu.co/index.php/nova/article/view/2687/2827
        4. Anele UA, Le B V, Resar LMS, Burnett AL. How I Treat priapism. Blood. 2015;125(23):3551-8. Disponible en: https://doi.org/10.1182/blood-2014-09-551887
        5. Sampaio P, Milena F, Costa P. The prevalence of priapism in children and adolescents with sickle cell disease in Brazil. Int J Hematol. 2012;95(1):648–51. Disponible en: https://doi.org/10.1007/s12185-012-1083-0
        6. Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and Impotence in Homozygous Sickle Cell Disease. Arch Intern M. 2015;140(11):1434–7. Disponible en: https://doi.org/10.1001/archinte.1980.00330220022011
        7. Wang CS, Kao WT, Chen CD, Tung YP, Lung FW. Priapism associated with typical and atypical antipsychotic medications. Int Clin Psychopharmacol [Internet]. 2006;21(4). Disponible en: https://journals.lww.com/intclinpsychopharm/fulltext/2006/07000/priapism_associated_with_typical_and_atypical.8.aspx
        8. Rodríguez Villalba R, García S, Puigvert Martínez A, María Pomerol I Montseny J, Munárriz R. Priapismo. Actas Urológicas Españolas [Internet]. 2005;29(10):961–8. Disponible en: https://doi.org/10.1016/S0210-4806(05)73377-5
        9. Wang Y, Zhang J, Li H. Narrative review: pathogenesis, diagnosis, and treatment of sleep-related painful erection [Internet]. Transl Androl Urol. 2021;10(12). Disponible en: https://tau.amegroups.org/article/view/86178
        10. Broderick GA. Priapism and Sickle-Cell Anemia: Diagnosis and Nonsurgical Therapy [Internet]. J Med. 2012;9(1):88–103. Disponible en: https://doi.org/10.1111/j.1743-6109.2011.02317.x
        11. Raafat M, Mohammed H. Priapism : Current Updates in Clinical Management ; Review article. [Internet] EJHM. 2022;88(1):3661–7. Disponible en: https://doi.org/10.21608/EJHM.2022.249512
        12. Bivalacqua, TJ; Allen, BK; Brock G et al. The diagnosis and management of recurrent ischemic priapism, priapism in sickle cell patients, and non-ischemic priapism: an AUA/SMSNA guideline [Internet]. J Urol. 2022;208(1):43–52.
        13. Ugwumba, FO, Ekwedigwe, HC, Echetabu KN, Okoh, AD, Nnabugwu, I, Ugwuidu E. Ischemic priapism in South ‑ East Nigeria : Presentation , management challenges , and aftermath issues [Internet]. Niger J Clin Pract. 2016;19:207–11. Disponible en: https://doi.org/10.4103/1119-3077.175968
        14. Aldallal S, Aldallal N, Alam A. Sickle cell-induced ischemic priapism [Internet]. Cogent Med. 2017;3(1):1268357. 10.1080/2331205X.2016.1268357
        15. Attar FS, Mohammad MA. Long-term outcome of sexual function in sickle cell disease men with ischemic priapism: A systematic review [Internet]. J Clin Urol. 2021;15(2):114–7. Disponible en: https://doi.org/10.1177/20514158211014051
        16. Okoko AR, Odzébé ASW, Moyen E, Ekouya Bowassa G, Oko APG, Mbika-Cardorelle A, et al. Priapism in children and adolescents with homozygous sickle cell disease in Brazzaville [Internet]. Prog Urol. 2014 Jan;24(1):57–61. Disponible en: https://doi.org/10.1016/j.purol.2013.04.021
        17. Arduini GAO, Marqui ABT De, Arduini GAO, Marqui ABT De. Prevalence and Characteristics of Priapism in Sickle Cell Disease [Internet]. Hemoglobin. 2018;42(2):73–7. Disponible en: https://doi.org/10.1080/03630269.2018.1452760
        18. Mougougou A, Ndang S, Milama N, Ngomas JF, Boumas N, Chimelle F, et al. Management of Priapism in Sickle Cell Patients: Experience of the Urology Department of the University Hospital Centre of Libreville [Internet]. Open J Urol. 2021;11(1):87–94. Disponible en: https://doi.org/10.4236/oju.2021.113009
        19. Idris IM, Burnett AL, Debaun MR. Epidemiology and treatment of priapism in sickle cell disease [Internet]. Hematology. 2022;2022(1):450–8. Disponible en: https://doi.org/10.1182/hematology.2022000380.
        20. Proudman RGW, Pupo AS, Baker JG. The affinity and selectivity of α-adrenoceptor antagonists, antidepressants and antipsychotics for the human α2A, α2B, and α2C-adrenoceptors and comparison with human α1 and β-adrenoceptors [Internet]. Pharmacol Res Perspect. 2022;10(2):e00936. Disponible en: https://doi.org/10.1002/prp2.936
        21. Greiner T, Schneider M, Regente J, Toto S, Grohmann R, Heinze M. Priapism induced by various Psychotropics: A Case Series [Internet]. World J Biol Psychiatry. 2019;20(6):505–12. Disponible en: https://doi.org/10.1080/15622975.2018.1520396
        22. Kamel K, Mohamed M, Abderrazek B, Mohamed C, Amine D, Riadh BS, et al. Prise en charge du priapisme à bas débit vue tardivement: à propos de 28 cas [Internet]. African J Urol. 2016;22(4):297–304. Disponible en: https://doi.org/10.1016/j.afju.2015.10.005
        23. Azbell RCG, Desai PC. Treatment dilemmas: strategies for priapism, chronic leg ulcer disease, and pulmonary hypertension in sickle cell disease [Internet]. Hematol Am Soc Hematol Educ Progr. 2021;1:411–7. Disponible en: https://doi.org/10.1182/hematology.2021000275
        24. Kato GJ, Mcgowan V, Machado RF, Little JA, Vi JT, Morris CR, et al. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease [Internet]. Blood. 2006;107(6):2279–85. Disponible en: https://doi.org/10.1182/blood-2005-06-2373.
        25. Vi JGT, Nolan VG, Mendelsohn L, Kato GJ, Gladwin MT, Steinberg MH. Chronic Hyper-Hemolysis in Sickle Cell Anemia : Association of Vascular Complications and Mortality with Less Frequent Vasoocclusive Pain [Internet]. PLoS One. 2008;3(5):e2095. Disponible en: https://doi.org/10.1371/journal.pone.0002095
        26. Birnbaum BF, Pinzone JJ. Sickle cell trait and priapism: a case report and review of the literature [Internet]. Cases J. 2008;1(1):7–9. 10.1186/1757-1626-1-429
        27. Helvaci MR, Gokce C, Davran R, Akkucuk S, Ugur M, Oruc C. Mortal quintet of sickle cell diseases [Internet]. Int J Clin Exp Med [Internet]. 2015;8(7):11442–8. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4565344/
        28. Yuan C, Quinn E, Kucukal E, Kapoor S, Umut A, Little JA, et al. Priapism, hemoglobin desaturation, and red blood cell adhesion in men with Sickle Cell Anemia [Internet]. Blood Cells Mol Dis. 2019;79:102350. Disponible en: https://doi.org/10.1016/j.bcmd.2019.102350.
        29. Adeyoju AB, Olujohungbe ABK, Morris J, Yardumian A, Bareford D, Akenova A, et al. Priapism in sickle-cell disease; incidence, risk factors and complications - An international multicentre study [Internet]. BJU Int. 2002;90(9):898–902. Disponible en: https://doi.org/10.1046/j.1464-410X.2002.03022.x
        30. Vilas C, Figueiredo B, Santiago RP, Conceic C, Neres S, Mateus A, et al. Priapism in sickle cell disease : Associations between NOS3 and EDN1 genetic polymorphisms and laboratory biomarkers [Internet]. PLoS One. 2021;16(2):e0246067. Disponible en: https://doi.org/10.1371/journal.pone.0246067
        31. Oluwagbemiga O. Adeodu, Morenike A. Akinlosotu SAA and SBAO. Foetal Haemoglobin and Disease Severity in Nigerian Children with Sickle Cell. Mediterr [Internet]. J Hematol Infect Dis. 2017;9(1):1–8. Disponible en: https://doi.org/10.4084/MJHID.2017.063
        32. Alvaia MA, Maia HAA da S, Nelli A de M, Guimarães COS, Carvalho ES de S, Netto JMB, et al. Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function [Internet]. Einstein (Sao Paulo). 2020;18:eAO5070. Disponible en: https://doi.org/10.31744/einstein_journal/2020AO5070
        33. Anele UA, Morrison BF, Burnett AL, Hopkins TJ. Molecular Pathophysiology of Priapism : Emerging Targets [Internet]. Curr Drugs Targets. 2015;16(5):474–83. Disponible en: https://doi.org/10.2174/1389450115666141111111842
        34. Kato GJ. Priapism in Sickle-Cell Disease: A Hematologist’s Perspective [Internet]. J Sex Med. 2012;9(1):70–8. Disponible en: https://doi.org/10.1111/j.1743-6109.2011.02287.x
        35. Nolan VG, Wyszynski DF, Farrer LA, Steinberg MH, Ben BEN, Car BEN, et al. Hemolysis-associated priapism in sickle cell disease data from the Cooperative Study for Sickle [Internet]. Blood. 2005;106(9):3264–7. Disponible en: https://doi.org/10.1182/blood-2005-04-1594.Supported
        36. Alkindi S, Almufargi SS, Pathare A. Clinical and laboratory parameters , risk factors predisposing to the development of priapism in sickle cell patients [Internet]. Biol Med. 2020;245(1):79–83. Disponible en: https://doi.org/10.1177/1535370219892846
        37. Idris IM, Abba A, Galadanci JA, Aji SA, Jibrilla AU, Rodeghier M, et al. Incidence and predictors of priapism events in sickle cell anemia: a diary-based analysis [Internet]. Blood Adv. 2022;6(20):5676–83. Disponible en: https://doi.org/10.1182/bloodadvances.2022007285
        38. Bello A, Maitama HY, Ahmed M, Sudi A. Surgical treatment of priapism in sickle cell patients: A noble therapy in preserving erection [Internet]. Arch Int Surg. 2016;6(1):12–6. Disponible en: https://doi.org/10.4103/2278-9596.187200
        39. Idris IM, Abba A, Galadanci JA, Mashi SA, Hussaini N, Gumel SA, et al. Men with sickle cell disease experience greater sexual dysfunction when compared with men without sickle cell disease [Internet]. Blood Adv. 2020;4(14):3277–83. Disponible en: https://doi.org/10.1182/bloodadvances.2020002062

        Information

        Directories and indexers
        Google Scholar profile

        QR code

        Keywords

        Most read in the last 30 days

        Current Issue

        Sistema OJS 3.4.0.7 - Metabiblioteca |