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Priapism in patients with hemolytic disorders: a nationwide retrospective cohort study

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Abstract

Priapism is a persistent, painful erection, which can lead to permanent penile damage and reduced quality of life. Patients with sickle cell disease have an increased risk of priapism which has been related to chronic hemolysis. This study investigates the prevalence of priapism in all major hereditary and acquired forms of hemolytic disorders. Patients with hemolytic disorders were identified in the nationwide Danish Hemolysis Cohort. Each patient was age-sex-matched with 50 comparisons from the general population without hemolysis. We identified the episodes of hospital-registered priapism events for both patients with hemolysis disorders and comparisons in the Danish National Patient Register between 1977 and 2016. We identified 4181 male patients with hemolytic disorders and 205,994 male comparisons, with 2,294,027 person-years of total observation time. Totally, 101 episodes of priapism occurred during follow-up period. Six episodes of priapism were recorded in three patients with a hemolytic disorder, all affected by sickle cell disease. Two of these patients had verified genotype HbSS. The incidence rate for first priapism in sickle cell disease was 432.8 per 100,000 person-years [95% CI: 139.6; 1341.8] versus 0.84 per 100,000 person-years [95% CI 0.54; 1.32] in comparisons. Using a large nationwide cohort, we found that only sickle cell disease is associated with priapism among patients with hemolytic disorders. The incidence rate of priapism in patients with sickle cell disease was lower than previously reported.

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References

  1. Shigehara K, Namiki M (2016) Clinical management of priapism: a review. World J Mens Health 34(1):1–8. https://doi.org/10.5534/wjmh.2016.34.1.1

    Article  PubMed  PubMed Central  Google Scholar 

  2. Cherian J, Rao AR, Thwaini A, Kapasi F, Shergill IS, Samman R (2006) Medical and surgical management of priapism. Postgrad Med J 82(964):89–94. https://doi.org/10.1136/pgmj.2005.037291

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Olujohungbe A, Burnett AL (2013) How I manage priapism due to sickle cell disease. Br J Haematol 160(6):754–765. https://doi.org/10.1111/bjh.12199

    Article  CAS  PubMed  Google Scholar 

  4. Becerra-Pedraza LC, Jiménez-Martínez LE, Peña-Morfin I, Nava-Esquivel R, Villegas-Martínez JA (2018) Priapism as the initial sign in hematologic disease: case report and literature review. Int J Surg Case Rep 43:13–17. https://doi.org/10.1016/j.ijscr.2017.12.038

    Article  PubMed  PubMed Central  Google Scholar 

  5. Alvaia MA, Maia HAAdS, Nelli AdM, Guimarães COS, Carvalho ESdS, Netto JMB, et al. Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function. Einstein (São Paulo). 2020;18.

  6. Melman A, Serels S (2000) Priapism. Int J Impot Res 12(Suppl 4):S133–S139. https://doi.org/10.1038/sj.ijir.3900592

    Article  PubMed  Google Scholar 

  7. Levey HR, Segal RL, Bivalacqua TJ (2014) Management of priapism: an update for clinicians. Ther Adv Urol 6(6):230–244. https://doi.org/10.1177/1756287214542096

    Article  PubMed  PubMed Central  Google Scholar 

  8. Eland IA, van der Lei J, Stricker BH, Sturkenboom MJ (2001) Incidence of priapism in the general population. Urology 57(5):970–972. https://doi.org/10.1016/s0090-4295(01)00941-4

    Article  CAS  PubMed  Google Scholar 

  9. Nolan VG, Wyszynski DF, Farrer LA, Steinberg MH (2005) Hemolysis-associated priapism in sickle cell disease. Blood 106(9):3264–3267. https://doi.org/10.1182/blood-2005-04-1594

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Kato GJ, Steinberg MH, Gladwin MT (2017) Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Invest 127(3):750–760. https://doi.org/10.1172/jci89741

    Article  PubMed  PubMed Central  Google Scholar 

  11. Adeyoju AB, Olujohungbe AB, Morris J, Yardumian A, Bareford D, Akenova A et al (2002) Priapism in sickle-cell disease; incidence, risk factors and complications - an international multicentre study. BJU Int 90(9):898–902. https://doi.org/10.1046/j.1464-410x.2002.03022.x

    Article  CAS  PubMed  Google Scholar 

  12. Serjeant G, Hambleton I (2015) Priapism in homozygous sickle cell disease: a 40-year study of the natural history. West Indian Med J 64(3):175–180. https://doi.org/10.7727/wimj.2014.119

    Article  CAS  PubMed  Google Scholar 

  13. Hansen DL, Overgaard UM, Pedersen L, Frederiksen H (2016) Positive predictive value of diagnosis coding for hemolytic anemias in the Danish National Patient Register. Clin Epidemiol 8:241–252. https://doi.org/10.2147/clep.S93643

    Article  PubMed  PubMed Central  Google Scholar 

  14. Hansen DL, Moller S, Andersen K, Gaist D, Frederiksen H (2019) Evans syndrome in adults - incidence, prevalence, and survival in a nationwide cohort. Am J Hematol 94(10):1081–1090. https://doi.org/10.1002/ajh.25574

    Article  PubMed  Google Scholar 

  15. Hansen DL, Möller S, Andersen K, Gaist D, Frederiksen H (2020) Increasing incidence and prevalence of acquired hemolytic anemias in Denmark, 1980–2016. Clin Epidemiol 12:497–508. https://doi.org/10.2147/clep.S250250

    Article  PubMed  PubMed Central  Google Scholar 

  16. Schmidt M, Pedersen L, Sørensen HT (2014) The Danish Civil Registration System as a tool in epidemiology. Eur J Epidemiol 29(8):541–549. https://doi.org/10.1007/s10654-014-9930-3

    Article  PubMed  Google Scholar 

  17. Schmidt M, Schmidt SA, Sandegaard JL, Ehrenstein V, Pedersen L, Sørensen HT (2015) The Danish National Patient Registry: a review of content, data quality, and research potential. Clin Epidemiol 7:449–490. https://doi.org/10.2147/clep.S91125

    Article  PubMed  PubMed Central  Google Scholar 

  18. Schmidt M, Schmidt SAJ, Adelborg K, Sundbøll J, Laugesen K, Ehrenstein V et al (2019) The Danish health care system and epidemiological research: from health care contacts to database records. Clin Epidemiol 11:563–591. https://doi.org/10.2147/clep.S179083

    Article  PubMed  PubMed Central  Google Scholar 

  19. Levine JF, de Tejada IS, Payton TR, Goldstein I (1991) Recurrent prolonged erections and priapism as a sequela of priapism: pathophysiology and management. J Urol 145(4):764–7. https://doi.org/10.1016/s0022-5347(17)38445-8

    Article  CAS  PubMed  Google Scholar 

  20. ICD-10: WHO international, https://www.who.int/classifications/icd/factsheet/en/. (1994-). Accessed.

  21. Steindel SJ (2010) International classification of diseases, 10th edition, clinical modification and procedure coding system: descriptive overview of the next generation HIPAA code sets. J Am Med Inform Assoc 17(3):274–82. https://doi.org/10.1136/jamia.2009.001230

    Article  PubMed  PubMed Central  Google Scholar 

  22. Hansen DL, Glenthøj A, Möller S, Biemond BJ, Andersen K, Gaist D et al (2020) Prevalence of congenital hemolytic disorders in Denmark, 2000–2016. Clin Epidemiol 12:485–495. https://doi.org/10.2147/clep.S250251

    Article  PubMed  PubMed Central  Google Scholar 

  23. 15 S. StataCorp STATA. 2017. p. Stata statistical software: release 15. College Station, TX: StataCorp LLC.

  24. StatisticsDenmark: Statbank. https://www.statbank.dk/. (2016). Accessed.

  25. Öz S, Küpeli S, Sezgin G, Bayram İ (2017) Thalassemia major and priapism: a case report of an adolescent. J Pediatr Hematol Oncol 39(6):e336–e337. https://doi.org/10.1097/mph.0000000000000846

    Article  PubMed  Google Scholar 

  26. Hacker HW, Schwoebel MG, Szavay PO (2018) Nonischemic priapism in childhood: a case series and review of literature. Eur J Pediatr Surg 28(3):255–260. https://doi.org/10.1055/s-0037-1599839

    Article  PubMed  Google Scholar 

  27. Mallat NS, Wehbe D, Haddad A, Cappellini MD, Marcon A, Koussa S et al (2014) Priapism, an emerging complication in β-thalassemia intermedia patients. Hemoglobin 38(5):351–354. https://doi.org/10.3109/03630269.2014.960087

    Article  CAS  PubMed  Google Scholar 

  28. De Rose AF, Mantica G, Tosi M, Bovio G, Terrone C (2016) Priapism and glucose-6-phosphate dehydrogenase deficiency: an underestimated correlation? Arch Ital Urol Androl 88(3):235–236. https://doi.org/10.4081/aiua.2016.3.235

    Article  PubMed  Google Scholar 

  29. Bartolucci P, Brugnara C, Teixeira-Pinto A, Pissard S, Moradkhani K, Jouault H et al (2012) Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis. Blood 120(15):3136–3141. https://doi.org/10.1182/blood-2012-04-424184

    Article  CAS  PubMed  Google Scholar 

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Author notes

  1. Stinne Tranekær and Dennis Lund Hansen contributed equally to the work

Authors and Affiliations

  1. The Research Unit of Hematology, Department of Clinical Research, University of Southern Denmark, Odense, Denmark

    Stinne Tranekær, Dennis Lund Hansen, Anne Lykke Sørensen & Henrik Frederiksen

  2. Department of Hematology, Odense University Hospital, J.B. Winsløws Vej 4, 5000, Odense, Denmark

    Dennis Lund Hansen & Henrik Frederiksen

  3. Department of Hematology, Amsterdam University Medical Centers, Amsterdam, The Netherlands

    Bart J. Biemond

  4. Department of Hematology, Rigshospitalet, Copenhagen, Denmark

    Andreas Glenthøj & Jesper Petersen

Authors
  1. Stinne Tranekær
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  2. Dennis Lund Hansen
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  3. Bart J. Biemond
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  4. Anne Lykke Sørensen
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  5. Andreas Glenthøj
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Contributions

DLH and HF conceived the idea for the study. ST, ALS, and DLH did the statistical analysis. ST wrote the first draft of the article and all seven authors contributed in writing subsequent drafts. All authors approved the final version of the manuscript.

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Correspondence to Dennis Lund Hansen.

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Tranekær, S., Hansen, D.L., Biemond, B.J. et al. Priapism in patients with hemolytic disorders: a nationwide retrospective cohort study. Ann Hematol 100, 1947–1951 (2021). https://doi.org/10.1007/s00277-021-04577-x

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  • DOI: https://doi.org/10.1007/s00277-021-04577-x

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