Priapism in Patients with Chronic Myeloid Leukemia (CML): A Systematic Review Priapism in Chronic Myeloid Leukemia

Main Article Content

Elrazi Ali
Ashraf Soliman
Vincenzo De Sanctis
Doris Nussbaumer
Mohamed Yassin

Keywords

Chronic myeloid leukemia, chronic granulocytic leukemia, Erectile dysfunction, Priapism, Male fertility

Abstract

Background: Priapism is defined as a penile erection that persists four or more hours and is unrelated to sexual stimulation. Priapism resulting from hematologic malignancy is most likely caused by venous obstruction from microemboli/thrombi and hyperviscosity caused by the increased number of circulating leukocytes in mature and immature forms. In patients with leukemia, 50% of cases of priapism are due to Chronic Myeloid Leukemia (CML). We present a systematic review of priapism in CML. Acquisition of evidence: An extensive literature research was carried out in PubMed, Google Scholar, SCOPUS, and Science Citation Index databases... The search included cases up to 4th August 2020. Synthesis of evidence: A total of 68 articles were found and included in our review, including 3 reviews from three different centers.  We found 68 articles (102 patients; figure 1) and several case reports on priapism in CML. Priapism was noticed in some patients at the first presentation of CML. However, it was infrequently reported during the start of treatment, following the stop of medication and post-splenectomy. The mean age at presentation was 27.4 years, and the mean time from onset of priapism to the time to get medical attention (presentation) was 78.2 hours. The mean white blood cell count associated with priapism was 321.29x109/L, and the mean platelet count was 569 x10 9/L. The chronic phase of CML was the most common phase where priapism occurred. Most patients were Asian (>50%). Nearly a quarter of patients (27.4%) developed permanent erectile dysfunction. Conclusions: Priapism is a urological emergency requiring urgent multidisciplinary management to prevent erectile dysfunction. Because of the relatively rare occurrence of priapism in CML patients, there is no standard treatment protocol.


 

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...
Abstract 21 |

References

1. Rodgers R, Latif Z, Copland M. How I manage priapism in CML patients. Br J Haematol 2012; 158: 155‑64.

2. Salonia A, Eardley I, Giuliano F, Hatzichristou D, Moncada I, Vardi Y, Wespes E, Hatzimouratidis K; European Association of Urology. European Association of Urology guidelines on priapism. Eur Urol. 201465:480-9.

3. Broderick GA, Kadioglu A, Bivalacqua TJ, Ghanem H, Nehra A, Shamloul R. Priapism: pathogenesis, epidemiology, and management. J Sex Med. 2010;7:476-500
4. Bennett N, Mulhall J. Sickle cell disease status and outcomes of African-American men presenting with priapism. J Sex Med. 2008;5:1244–50.
5. Turkina A, Wang J, Mathews V, Saydam G, Jung CW, Al Hashmi HH, et al . TARGET: a survey of real‐world management of chronic myeloid leukaemia across 33 countries. Br J Haematol. 2020;190: 869-76.
6. Mulhall JP, Honig SC. Priapism: etiology and management. Acade Emerg Med. 19963:810-6.
7. Hashmat AI, Rehman JU: Priapism. In: Hashmat AI, Das S (eds.), The Penis. Philadelphia, Lea & Febiger. 1993; pp. 219-43.
8. Yassin MA, Soliman AT, Sanctis VD. Effects of tyrosine kinase inhibitors on spermatogenesis and pituitary gonadal axis in males with chronic myeloid leukemia. J Cancer Res Ther. 2014;2:116-21.
9. Yassin MA, Abdulla MA-J, Chandra P, et al. Chronic Myeloid Leukemia in Adolescents and Young Adults: A Single Institute Experience. Blood. 2019;134 (Supplement 1):5915.
10. Ergenc H, Varım C, Karacaer C, Çekdemir D. Chronic myeloid leukemia presented with priapism: Effective management with prompt leukapheresis. Niger J Clin Pract. 2015;18:828-30.


11. Thakur P, Verma V, Fotedar V, Singh K. Priapism in a Pediatric Chronic Myeloid Leukaemia Patient: Unusual Presentation of a Rare Disease in Children Case Report. Clin Cancer Investig J. 2019;8:76.
12. Dhar J, Chhabra G, Khandelwal L, Batra A, Gupta N. Priapism as a debut presentation of chronic myeloid leukemia. J Coll Physicians Surg Pakistan. 2019;29:78–80.
13. Ponniah A, Brown CT, Taylor P. Priapism secondary to leukemia: Effective management with prompt leukapheresis. Int J Urol. 2004;11:809–10.
14. Anjum F FS. Chronic Myeloid Leukemia Presenting with Priapism. J Leuk. 2015; 3: 171.
15. Becerra-Pedraza LC, Jiménez-Martínez LE, Peña-Morfin I, Nava-Esquivel R, Villegas-Martínez JA. Priapism as the initial sign in hematologic disease: Case report and literature review. Int J Surg Case Rep. 2018;43:13–7.
16. Htun TH, Dublin N, Parameswaran M, Razack AH, Chua CB. Chronic myeloid leukaemia presenting as Priapism - how should We treat these? J Heal Transl Med. 2008;11:27–9.
17. Ocheni S, Ibegbulam O, Olusina D, Oyekunle A, Durosinmi M. Chronic myeloid leukaemia presenting as priapism: a report of 2 cases and review of literature. Int J Med Health Dev.2010;15:76-81.
18. Afrose R, Nebhnani D, Wadhwa N. Cutaneous myeloid sarcoma of the penile foreskin. Turk Patoloji Derg. 2015;31:131-5.
19. Jana K, Aggarwal R, Gawande A, Lal M. Priapism: A chronic myeloid leukemia harbinger in exigency. Ann Trop Med Public Health. 2013;6:583–5.
20. Dhanju AS, Tyagi P, Dhaliwal SS, et al. Priapism: a rare presentation in chronic myeloid leukemia. Int J Adv Med.2019;6:1937-9.
21. Aggarwal V, Himanshu, Sathi S, Gupta A, Agrawal P. Priapism in CML. Indian J Med Paediatr Oncol. 2008;29:30-1.
22. Sareen R, Kapil M, Malpani BK. Priapism: A Rare Presentation of CML. J Hematol Oncol Forecast.2018;1:1-3.


23. Chang M-W, Tang C-C, Chang SS. Priapism--a rare presentation in chronic myeloid leukemia: case report and review of the literature. Chang Gung Med J. 2003;26:288-92.
24. Huei TJ, Lip HTC, Omar S. A rare presentation of chronic myeloid leukaemia with priapism treated with corporoglandular shunting. Med J Malaysia. 2018;73:420–2.
25. Wajih Ullah M, Rehman A, Cheeti A, et al.Priapism and chronic myelogenous leukemia Int J Adv Res.2018;6:144-6.
26. Shaeer OK, Shaeer KZ, AbdelRahman IF, El-Haddad MS, Selim OM. Priapism as a result of chronic myeloid leukemia: Case report, pathology, and review of the literature. J Sex Med. 2015;12:827–34.
27. Becker HC, Pralle H, Weidner W. Therapy of priapism in high counting myeloid leukemia - A combined oncological-urological approach. Two case reports. Urol Int. 1985;40:284–6.
28. Qu M, Lu X, Wang L, Liu Z, Sun Y, Gao X. Priapism secondary to chronic myeloid leukemia treated by a surgical cavernosa-corpus spongiosum shunt: Case report. Asian J Urol. 2019;6:373–6.
29. SwapnaY, Narmada N. Emergency leukapheresis in chronic myeloid leukemia presenting with priapism. Asian J Pharm Heal Sci.2017:7:1701-4.
30. Abd El Salam M, Ibrahim NH, Hassan S. Discontinuation of treatment in a chronic myeloid leukemia patient caused priapism: A case report. Hum Androl. 2019;9:21–3.
31. Atas U, Meydanal YE, Iltar U, Ulas T, Salim O, Undar L. Priapism-A Rare Presentation of Chronic Myeloid Leukaemia. J Clin Diagnostic Res. 2019;13: DOI : 10.7860/JCDR/2019/39817.12612
32. Dogra PN, Kumar P, Goel R, Dash SC. Long duration priapism in blast crisis of chronic myeloid leukemia. J Ass Phys India. 2004;52:170.
33. Jameel T, Mehmood K. Priapism – an unusual presentation in chronic myeloid leukaemia: case report and review of the literature. Biomedica. 2009;25:197–9.
34. Irzi M, MhannaT, El Houmaidi A, et al. Delayed penile prosthesis implantation in the delayed presentation of ischemic priapism. Arch Case Reports. 2020;4(1):004–006.

35. Tazi L. Priapism as the first manifestation of chronic myeloid leukemia. Ann Saudi Med. 2009; 29:412.
36. Khan A, Shafiq I, Shah MH, Khan S, Shahid G, Arabdin M. Chronic myeloid leukaemia presenting as priapism: A case report from Khyber Pakhtunkhwa. J Pak Med Assoc. 2018;68:942–4.
37. Patil RB, Wasekar N, Bamborde S, et al. Priapism: Rare presenting manifestation of chronic myeloid leukemia and its management-Case series of 5 patients. Int J Sci Res. 2019;8:35-7.
38. Hazra SP, Priyadarshi V, Gogoi D, Sharma PK, Pal DK, Chakraborty SC. Pediatric priapism: a rare first manifestation of leukemia. APSP J Case Rep. 2013;4:39.
39. Veljković D, Kuzmanović M, Mićić D, Šerbić-Nonković O. Leukapheresis in management hyperleucocytosis induced complications in two pediatric patients with chronic myelogenous leukemia. Transfus Apher Sci. 2012;46:263–7.
40. Ammouri Z, Mouhaoui M. Priapism a rare and unusual presentation in chronic myeloid leukemia [a case report].AMMUR .2019;3:36.
41. Castagnetti M, Sainati L, Giona F, Varotto S, Carli M, Rigamonti W. Conservative management of priapism secondary to leukemia. Pediatr Blood Cancer. 2008 ;51:420–3.
42. Avci AE, Kurtulus F, Fazlioglu A, Keskin S, Güçtaş Ö, Cek M. Priapism as an initial presentation of chronic myelogenous leukemia: A case report. UHOD. 2005;15: 153-5.
43. Morano SG, Latagliata R, Carmosino I, Girmenia C, Dal Forno S, Alimena G. Treatment of long-lasting priapism in chronic myeloid leukemia at onset. Ann Hematol. 2000;79:644–5.
44. Kumar P, Rahman K, Kumari S, Singh MK, Gupta R, Nityanand S. Priapism as a rare presentation of chronic myeloid leukemia. J Can Res Ther. 2018;14:1442-3.
45. Graivier L, Gran G, Rhoades RB, Reynolds RC, Windmiller J. Priapism in a 7-week-old infant with chronic granulocytic leukemia. J Urol. 1971;105:137–9.
46. Patil P L , Somkuwar K , Katariya P S, Gaikwad N. Priapism - A Rare Presentation in Chronic Myeloid Leukemia. Vidarbha J Intern Med.2016;21:50-1.

47. Agarwal A, Lavania P. Priapism in a patient of chronic myeloid leukemia: A case report. Indian J Urol. 2020;24:S53.
48. Ritesh Chaudhary, Bijendra Kumar Rai, Rabin Bhandari, Ajay Yadav. Unusual Case of Priapism in Emergency Department of Tertiary Care Hospital of Eastern Nepal. Int J Clin Urol. 2017;4:39-40.
49. Shafique S, Bona R, Kaplan AA. A case report of therapeutic leukapheresis in an adult with chronic myelogenous leukemia presenting with hyperleukocytosis and leukostasis. Ther Apher Dial. 2007;11:146–9.
50. Stutz FH, Bergin JJ. Priapism in Leukemia: A report of two cases. Mil Med. 1970; 135:44–8.
51. Suri R, Goldman JM, Catovsky D, Johnson SA, Wiltshaw E, Galton DAG. Priapism complicating chronic granulocytic leukemia. Am J Hematol. 1980;9:295–9.
52. Chowdhury ZZ, Al-Asad H, Rahman MH, et al. Management of Priapism with Chronic Myeloid Lukaemia- A Rare Presentation and Our Experiences. Haematol J Bangladesh. 2020;3:39–41.
53. Nabi G, Dogra PN. Re: Chronic myeloid leukaemia presenting as priapism in children: need for multidisciplinary approach. East Afr Med J. 2000;77:576.
54. Gupta A, Bambrey P, Varma S, Vaidyanathan S, Deodhar SD. Priapism in chronic myeloid leukaemia: combined medical and surgical treatment. A report on two patients. Indian J Cancer. 1987;24:176-9.
55. Dutta TK, Purohit OP, Vaidyanathan V, Gupta BD, Rao MS. Radiation therapy of priaprism complicating chronic myeloid leukaemia--review and report of a case. Indian J Cancer.1979;16:90-3.
56. Ekeke O, Omunakwe H, Nwauche C. Chronic myeloid leukaemia presenting as priapism. Int Surg. 2015;100:552–7.
57. Almaeena W, Azzuz S.Undiagnosed Chronic Myelogenous Leukemia Presented By Priapism. Int J Acad Sci Res.2020;4:20-1.
58. Ritz ND, Purfar M. Chronic myeloid leukemia with priapism in eight-year-old child. N Y State J Med. 1964;64:553-6.

59. Babel CS, Jain KC, MathurA, Bhu N. Priapism in child with chronic granulocytic leukemia. Indian Pediatr.1976;13:961.
60. Agrawal DK, Jha S, Verma A, Tripathi AK, Singh BN. Priapism, complicting chronic myeloid leukemia. A case report. Indian J Cancer.1991;28:51-2.
61. Ghalaut PS, Kalra GS, Gupta S. Priapism - A rare presentation in chronic myeloid leukaemia. J Assoc Physicians India. 1996;44:354-5.
62. Bhatia P, Arya LS, Chinnappan D, Choudhry VP, Pati H. Priapism in chronic myelogenous leukemia. Indian J Pediatr. 1992;59:130-2.
63. Mishra K, Jandial A, Singh V, Radotra B, Malhotra P. Priapism in chronic myeloid leukemia: Meeting at the crossroads and heading in different directions. Indian J Med Paediatr Oncol. 2020;41:418.
64. Saikia T, Advani SH, Dinshaw KA, Gopal R, Nair CN, Chandwani IM. Priapism complicating chronic myeloid leukaemia and its management. J Indian Med Assoc. 1984;82:294-6.
65. Villegas Osorio JF, Corchuelo Maíllo C, Cuevas Palomino A, Medina López RA. Ischaemic priapism as a presentation of chronic myeloid leukaemia. Arch Esp Urol. 2014;67:708-11.
66. Clark AJ, Hsu P, Darves-Bornoz A, Tanaka ST, Mason EF, Katzenstein HM. Priapism in a 13-year-old boy. Pediatr Rev. 2018;39:617-9.
67. Haar H, Shanbrom E, Miller S. The treatment of leukemic priapism with A-139. J Urol. 1960;83:429-32.
68. Shankar J. Priapism in Teenager Chronic Myelogenous Leukemia: a Rare Occurrence. Asian J Pharm Heal Sci. 2011;1:226.
69. Graw RG, Skeel RT, Carbone PP. Priapism in a child with chronic granulocytic leukemia. J Pediatr.1969;74:788–90.
70. Abbott LS, Moineau G, Johnston DL. Case 1: An unusual cause of headaches and priapism in a teenager. Paediatr Child Health. 2008;13:299–301.
71. Ocheni S, Ibegbulam O, Olusina D, Oyekunle A, Durosinmi M. Chronic myeloid leukaemia presenting as priapism: a report of 2 cases and review of literature. J Coll Med. 2012;15:76–81.
72. Musa A, Ndakotsu M, Abubakar S, Agwu P. Chronic myeloid leukemia with an initial presentation as ischemic priapism: A case report and review of literature. Arch Int Surg. 2017;7:68.
73. Gupta A, Seth T, Gupta A. Successful use of terbutaline in persistent priapism in a 12-year-old boy with chronic myeloid leukemia. Pediatr Hematol Oncol. 2009;26:70-3.
74. Ervie M, Boongaling DC, Rose S, Mortel C, Deala RP. Priapism as a Rare Presentation of Chronic Myelogenous Leukemia. Philippine J Inter Med. 2015;53:1-5.
75. Nerli RB, Magdum PV, Hiremath SC, et al. Priapism - A Rare Presentation in Chronic Myeloid Leukemia: Case Report. Urology Case Reports. 2016;4:8-10. DOI: 10.1016/ j.eucr.2015.08.005.
76. Sun HH, Zhang JH, DeWitt-Foy M, Waldron M, Mukherjee S, Montague DK. Urologic Management of Priapism Secondary to Chronic Myeloid Leukemia. Urology. 2019;125:24–8.
77. Minckler MR, Conser E, Figueroa JJ, Scott AJ, Gaither J, Amini R. The Semantics of Priapism and the First Sign of Chronic Myeloid Leukemia. Case Rep Emerg Med. 2017;2017:2656203. doi: 10.1155/2017/2656203.
78. Savage DG, Szydlo RM, Goldman JM. Clinical features at diagnosis in 430 patients with chronic myeloid leukaemia seen at a referral centre over a 16-year period. Br J Haematol. 1997;96:111-6.
79. Steinhardt GF, Steinhardt E. Priapism in children with leukemia. Urology. 1981 ; 18:604-6.
80. Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and Impotence in Homozygous Sickle Cell Disease. Arch Intern Med. 1980;140:1434-7.
81. Jandial A, Mishra K, Sandal R, Lad D, Prakash G, Khadwal A, et al. CML patients presenting with priapism: Is there any disparity in outcome? J Clin Oncol. 2019;37 (15 Suppl): e18545- DOI:10.1200/JCO.2019.37.15_suppl.e18545.
82. Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood. 2002;100:2292–302.
83. Cherian J, Rao AR, Thwaini A, Kapasi F, Shergill IS, Samman R. Medical and surgical management of priapism. Postgrad Med J. 2006;82:89-94.
84 Faderl S, Talpaz M, Estrov Z, O’Brien S, Kurzrock R, Kantarjian HM. The biology of chronic myeloid leukemia. New Engl J Med. 1999;341:164–72.
85. Pfirrmann M, Baccarani M, Saussele S, et al. Prognosis of long-term survival considering disease-specific death in patients with chronic myeloid leukemia. Leukemia. 2016;30:48-56.
86. Pryor JP, Hehir M. The management of priapism. Br J Urol.1982;54:751-4.
87. Kumar M, Garg G, Sharma A, Pandey S, Singh M, Sankhwar SN. Comparison of outcomes in malignant vs. non-malignant ischemic priapism: 12-year experience from a tertiary center. Turkish J Urol. 2019;45:340-4.
88. Chang X, Zhou L, Chen X, et al. Impact of Imatinib on the Fertility of Male Patients with Chronic Myelogenous Leukaemia in the Chronic Phase. Target Oncol. 2017; 12: 827-32.
89. Montague DK, Jarow J, Broderick GA,et al.; Members of the Erectile Dysfunction Guideline Update Panel; Americal Urological Association. American Urological Association guideline on the management of priapism. J Urol. 2003;170:1318-24.
90. Kulmala RV, Tamella TL. Effects of priapism lasting 24 hours or longer caused by intracavernosal injection of vasoactive drugs. Int J Impot Res.1995;7:131-6.
91. Burnett AL, Sharlip ID. Standard Operating Procedures for Priapism. J Sex Med. 2013;10:180-94.
92. Ralph DJ, Garaffa G, Muneer A, et al. The Immediate Insertion of a Penile Prosthesis for Acute Ischaemic Priapism. Eur Urol. 2009;56:1033-8.