Case Report: Severe ischemic priapism as the clinical presentation in patient with chronic myeloid leukemia (CML) treated by proximal shunt

Dahril Dahril; Sangaji Ramadhan

doi:10.12688/f1000research.133785.1

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Case Report

Case Report: Severe ischemic priapism as the clinical presentation in patient with chronic myeloid leukemia (CML) treated by proximal shunt

[version 1; peer review: 1 approved, 1 approved with reservations]

Dahril Dahril ¹, Sangaji Ramadhan¹

PUBLISHED 20 Nov 2023

Author details Author details

¹ Urology, Universitas Padjadjaran, Bandung, West Java, 40161, Indonesia

Dahril Dahril
Roles: Conceptualization, Formal Analysis, Methodology, Project Administration, Supervision, Validation, Writing – Review & Editing

Sangaji Ramadhan
Roles: Data Curation, Investigation, Resources, Visualization, Writing – Original Draft Preparation

OPEN PEER REVIEW

REVIEWER STATUS

Abstract

Background: Priapism refers to an erection of the penis that persists for four hours or more and is not associated with sexual arousal or stimulation. This condition is thought to be caused by abnormal production of blood cells in the bone marrow, which leads to the release of certain chemicals into the bloodstream. The current article presents a severe ischemic priapism case, and assesses and contrasts the documented instances of patients with chronic myelogenous leukemia (CML) who experienced priapism as their primary clinical symptom over the last two decades.

Case presentation: The 21-year-old male patient presented with persistent and painful erections for three days. Further tests revealed he had CML and ischemic priapism. He was treated with distal shunting and proximal shunting with cytoreductive therapy to resolve the symptoms.

Conclusions: Priapism is a medical emergency that can cause permanent damage to the erectile tissue if not treated promptly. Prolonged and painful erections can cause ischemia, leading to tissue damage and decreased function. If priapism is caused by an underlying medical condition; delaying treatment can allow the underlying condition to progress and become more difficult to treat.

Keywords

Priapism, Chronic myeloid leukemia, Distal shunting, Proximal shunting

Corresponding author: Dahril Dahril

Competing interests: No competing interests were disclosed.

Grant information: The author(s) declared that no grants were involved in supporting this work.

Copyright: © 2023 Dahril D and Ramadhan S. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

How to cite: Dahril D and Ramadhan S. Case Report: Severe ischemic priapism as the clinical presentation in patient with chronic myeloid leukemia (CML) treated by proximal shunt [version 1; peer review: 1 approved, 1 approved with reservations]. F1000Research 2023, 12:1487 (https://doi.org/10.12688/f1000research.133785.1) First published: 20 Nov 2023, 12:1487 (https://doi.org/10.12688/f1000research.133785.1) Latest published: 20 Nov 2023, 12:1487 (https://doi.org/10.12688/f1000research.133785.1)

Introduction

Priapism is a medical condition characterized by prolonged penile erection lasting more than four hours; hematological disorders are one of its major causes. Chronic myeloid leukemia (CML) is the most frequent hematological disorder that can lead to priapism, and it is often accompanied by other symptoms such as hyperleukocytosis, lymphadenopathy, asthenia, and enlargement of the spleen and liver.¹^,² Prolonged priapism can lead to permanent erectile dysfunction, which is considered a urologic emergency.³

CML is a type of blood cancer caused by a chimeric oncogene, BCR-ABL, and can spread in both the myeloid and lymphoid lineages.⁴^,⁵ It is estimated to include 1-2 cases per 100,000 people each year, with an average age of 45 to 55 years old and 20% of adult leukemia cases. Symptoms include fatigue, weight loss, abdominal discomfort, bleeding, purpura, enlarged spleen, excess of white blood cells, anemia, and elevated platelet count.⁶ Priapism is a medical emergency that occurs when a man has an erection that lasts too long, not related to sexual arousal, and may not be relieved by ejaculation. Untreated, low-flow priapism may lead to permanent impotence.⁷^,⁸

Case report

A 21-year-old man came with a complaint of continuous penis erection for the past three days with an Erection Hardness Score (EHS) of 4. There was no fever or history of trauma, but the patient had been regularly consuming weight gain medication for the past two months. During the physical examination, the patient appeared to be weak and had an axilla temperature of 37°C. The eye examination revealed a pale conjunctiva as well as swelling and ulcers, and the examination of the genitalia showed a painful erection (Figure 1). The patient has agreed for the data and photos to be used in research and publication. The patient has signed a written informed consent.

Figure 1. The genitalia showed a painful erection (three days).

Laboratory tests showed WBC = 551×10³ /μL, Hb = 8.8 g/dL, hematocrit = 24%, MCV of 86 fL, MCH of 32 pg, MCHC of 30.80, and platelets count of 385×10³/μL. A differential count results were 87/87/3/3/5 ×10³/μL. PT=16.3s APTT = 30 s. A blood gas analysis showed pH: 6.34; pCO₂: 71.3 mmHg; pO₂: 11.43 mmHg; HCO₃ - : 4.83; SO₂c: 1.8%; TCO₂: 6.90 mmol/L.

The peripheral blood test showed anisocytosis and mild poikilocytosis and granulocytic series with 5% blast rate (Figure 2). The patient was suspected to have Chronic Myelocytic Leukemia (CML) and priapism, based on clinical and supporting information. A doppler ultrasonography revealed hypoechoic areas in the right and left corpora carvernosa with low flow in the right and left carvenosa arteries (Figure 3).

Figure 2. Peripheral blood smear results suggesting a hematologic malignancy.

Figure 3. Ultrasonography (USG) and Doppler Ultrasound (US) results showing low-flow priapism with penile edema.

The patient complained of pain and swelling, so it was decided to perform a Winter procedure in the operating room, with local anesthetic injected and a large core biopsy needle inserted through the glans and directed proximally to the corpus cavernosum (Figure 4).⁹

Figure 4. The patient after Winter’s procedure (distal shunt).

The glans penis became edematous and cyanotic after the Winter procedure and proximal shunting and debridement was performed, with a longitudinal shaft penis incision, dissection of the bulbocavernosus muscle, and parallel incisions in the corporal bodies (Figure 5).

Figure 5. The patient then underwent surgery through the proximal shunting.

Two days after proximal shunting, EHS dropped from 3 to 1, but edema persisted. Hydroxyurea therapy resulted in decreased leukocyte from 551,000 to 272,000. Reverse transcription multiplex PCR genetic testing revealed BCR-ABL (+) in the form of b2A2 and b3a2 fusion, encoding protein p210 or major breakpoint (Figure 6). Imatinib was given as CML therapy for the lifetime.

Figure 6. The patient after proximal shunting.

Discussion

Chronic myeloid leukaemia (CML)

CML is a type of blood cancer that occurs due to a genetic mutation caused by the fusion of two normal chromosomes.²^,¹⁰ It is common globally, with an average age of 40-60 years and a slightly higher occurrence in males (1.4:1).²^,¹¹ CML is classified as a type of myeloproliferative neoplasm. In CML, there is an excessive production of mature granulocytes. The disease progresses through three stages: the chronic phase, the accelerated phase, and the blast phase.¹² Diagnosis is often delayed due to vague clinical symptoms. Leukocytosis affects all levels of differentiation and maturation of myeloid cells, resulting in the overproduction of mature granulocytes, eosinophils, and basophils. The presence of the Philadelphia chromosome, which is a translocation of chromosomes 9 and 22, is seen in about 95% of CML cases (Table 1).¹³

Table 1. Overview of case reports and reviews.

First author	Year of publication
Ali¹	2021	The prompt administration of CML treatment may facilitate the prompt resolution of priapism and may be essential for achieving complete remission of the condition.
Chowdhury²⁶	2020	Six patients with CML were treated with minimally invasive procedures and one required a proximal shunt, all referred to a hematologist for proper management.
Jandial²⁷	2019	Treatment response was favorable, but long duration of symptoms and leukocytosis likely led to ischemic priapism and ED.
Kumar and Garg²⁸	2018	On average, individuals who presented with ischemic priapism caused by CML were 27.50 years old, mean duration was 4.25 days, no oncological treatment, eight successful detumescence.
Tendulkar²⁹	2017	Leukapheresis was successful in reducing white blood cell count and symptoms of leukostasis, with 1.6 sessions needed.
Ekeke³⁰	2015	Priapism was diagnosed in two patients with CML, but treatment methods were not specified.
Kurosawa³¹	2015	Imatinib and hydroxycarbamide treatment may have resulted in erectile dysfunction in two CML patients.
Pal³²	2015	Four CML patients experienced priapism after treatment with imatinib, hydroxycarbamide, leukapheresis, and aspiration or irrigation. It is possible that two CML patients developed erectile dysfunction after being treated.
Nabi³³	2000	The seven patients had successful detumescence after treatment with Winter shunts, hydroxycarbamide, and allopurinol, but none had potency during follow-up.

Abbreviations: CML, chronic myeloid leukemia; ED, erectile dysfunction; SD, standard deviation; WBC, white blood cell.

Priapism

Rapid treatment is necessary for priapism, which is a medical emergency and occurs at a rate of 1.5 cases per 100,000 person per years.¹⁴ It is a rare presentation of CML, affecting males aged 5-10 and 20-50, with hematological conditions as the main cause.²

Classification of priapism

Priapism is classified into three types based on its cause and how it affects the body: ischemic, nonischemic, and stuttering. The defining features of ischemic priapism include rigidity of the corpora cavernosa and inadequate blood flow to the cavernous arteries, leading to stiffness and pain.¹³ It can cause erectile dysfunction in up to 90% of cases, making it a medical emergency that must be treated promptly.¹⁵ Nonischemic priapism can be caused by trauma or congenital malformations, iatrogenic causes, or shunt procedures.¹⁶ Unlike ischemic priapism, nonischemic priapism is not considered a medical emergency and typically does not require immediate intervention.¹⁵ Stuttering priapism can cause permanent damage to the corpus cavernosum and erectile dysfunction,¹⁷ so it is important to treat it immediately with medication that addresses the underlying cause.¹⁵^,¹⁸

Pathophysiology of priapism

Theories on the origin of priapism are being revised, with the hypothesis that it may stem from dysregulation of nitric oxide (NO) in the blood vessels of the penis.¹⁹ Other possible causes include elevated adenosine levels and the influence of opiorphins.² The sludging of blood within the corpora cavernosa is the underlying cause of ischemic priapism, which leads to tissue ischemia and a reduction in oxygen supply to the smooth muscles.¹⁹^–²¹ Platelets begin to attach to the basement membrane of the sinusoidal endothelium after 12 hours, while cavernosal smooth muscle necrosis sets in after 48 hours.² Blood exchange transfusions have been shown to be effective and safe in treating patients with sickle cell disease and severe priapism (Table 2).¹

Table 2. Instances of priapism and leukemia have been reported in the past two decades.

Author (s)	Age (years) and diagnosis	Duration of the complaint	White blood cells	Signs, symptoms, and treatment
Author (s)	Age (years) and diagnosis	Duration of the complaint	10³ cell/mm³	Signs, symptoms, and treatment
Gaye, Thiam et al. 2020³⁴	46, Chronic myeloid leukemia	48 hours	526	- Hepatosplenomegaly - Puncturing the corpus cavernosum, and administering phenylephrine solution alongside the medications (i.e., hydroxiurea and imatinib)
Dhar, Chhabra et al. 2019³⁵	52, Chronic myeloid leukemia	4 hours	239	- Anaemic conjunctivae, hepatomegaly, and splenomegaly. - Winter's procedure, aspiration failure, imatinib, and hydroxyurea
Qu, Lu et al. 2018³⁶	18, Chronic myeloid leukemia	1 week	257	- Enlargement of liver and spleen. - Phenylephrine injection to the corpus cavernosum, aspiration failure - Winter's procedure, and use of the medication imatinib.
Becerra, Jimenez et al. 2018³⁷	52, Chronic myeloid leukemia	6 days	282	- General fatigue, pale appearance, unintended loss of weight. - Drainage and irrigation of the corpus cavernosum; and surgical penile shunts.
Khan, Shafiq et al. 2018³⁸	16, Chronic myeloid leukemia	11 days	614	- Splenomegaly, hepatomegaly, and a general paleness. - Draining and irrigating the corpora cavernosa, as well as undergoing a Winter’s procedure. Injecting the hydroxyurea medication.
Nerli, Magdum et al. 2016³⁹	19, Chronic myeloid leukemia	24 hrs	296	- Anaemic conjunctivae, enlargement of liver and spleen. - Irrigation and aspiration of the corpus cavernosa using phenylephrine solution. - Additional injected medications such as hydroxiurea and imatinib.
Shaeer, Shaeer et al. 2015⁴⁰	21, Chronic myeloid leukemia	6 days	410	- Dyspepsia’s symptoms, hepatomegaly, and splenomegaly - Phenylephrine injection, aspiration failure, prosthetic penis, and imatinib to the corpus cavernosa
Farhan, Anjum et al. 2015⁴¹	38, Chronic myeloid leukemia	30 hrs	155	- Abdominal discomfort, weight loss, hepatosplenomegaly, and pale conjunctivae - Administering the phenylephrine through punctures to the corpus cavernosum, hydroxiurea, and imatinib; aspiration failure.
Gupta, Seth, Gupta 2009⁴²	12, Chronic myeloid leukemia	2 days	346	- General paleness, enlargement of spleen and liver - Hydroxyurea, terbutaline, and imatinib.
Jameel and Mehmood 2009⁴³	21, Chronic myeloid leukemia	8 hrs	316	- Hepatosplenomegaly, anaemic conjunctivae, abdominal discomfort, weight loss, nosebleed. - Puncturing the corpus cavernosum using phenylephrine, aspiration failure; hydroxiurea.
Jameel and Mehmood 2009⁴³	55, Chronic myeloid leukemia	12 hrs	282	- Pale conjunctival mucosa. - Aspiration and taking hydroxiurea medication.
Ponniah, Brown and Taylor 2004⁴⁴	19, Chronic myeloid leukemia	18 hours	513	- Asymptomatic - Aspiration failure and leukapheresis. Treatment for CML was not available.
Chang et al. 2003⁴⁵	21, Chronic myeloid leukemia	not available	217	- Weight loss, enlarged spleen and liver, anaemic conjunctivae, and profuse bleeding. - Phenylephrine-induced corpus cavernosum puncture, aspiration failure, hydroxiurea, and interferon-2

Note: All patients on this table list have painful priapism.

Surgical management of priapism

Surgery is the first course of treatment to drain deoxygenated blood from the corpora cavernosa.

Guidelines recommend an aggressive approach for managing patients with refractory priapism. This typically involves a sequential approach starting with distal to proximal shunts, and then progressing to vein-shunting as needed. The goal is to resolve the priapism rapidly and safely. Distal corporoglanular shunts are intended to alleviate compartment syndrome by releasing blood that has become confined within the corpora. In addition to the sequential shunting approach, there are alternative surgical procedures for managing priapism. These may include the Ebbehoj shunt, the T-shunt with or without intracavernous tunneling, and the Al-Ghorab shunt. The Al-Ghorab shunt involves removing a portion of the tunica albuginea from the bilateral apex of the corpora cavernosa to drain blood from the penis and reduce the risk of sudden shunt closure. Pulmonary embolism and local thrombus development can make vein shunts more difficult to manage, and proximal or vein shunts have greater rates of erectile dysfunction. The duration of priapism is an important factor in the development of erectile dysfunction.

Priapism in CML patients

Priapism in leukemia is caused by a buildup of white blood cells that slows down blood flow, disrupting the balance of nitric oxide and cGMP, leading to an occlusion and prolonged lack of blood flow.¹ Before the availability of tyrosine kinase inhibitors, 85% of CML diagnoses were in the chronic phase, with the majority of patients being under 40 years and having a mean age of 27.4 years.²²^,²³ In adults without CML, the incidence of priapism is highest between 20-50 years of age, and up to 30% of these patients may have a platelet count above 600 × 10⁹/L. However, in CML patients with stuttering priapism, the platelet count is usually lower.²³ Ethnicity is significant, with 57% of cases being reported in Asian individuals, and priapism has been reported in patients who have compliance issues or who have stopped taking their medication.¹ It is a serious urologic condition that requires prompt treatment to prevent erectile dysfunction.

CML patients with priapism typically seek medical attention after an average of 78.28 hours, which increases their risk of developing erectile dysfunction.²⁴ Imatinib, a first-line tyrosine kinase inhibitor, has improved the prognosis, but has been shown to reduce sperm density, count, survival rates, and activity in chronic phase. Urological aspiration and irrigation should be used instead of oral medication, and leukapheresis can be used for both purposes.²⁵ Four techniques are used to treat priapism: percutaneous distal shunts, open proximal shunts, and vein anastomoses/shunts. Guidelines recommend taking an aggressive approach by proceeding with distal to proximal to vein shunting in a quick and safe manner to restore penile flaccidity.¹^,²⁵ Erectile dysfunction is more likely after proximal or vein shunts, but it’s challenging to attribute the dysfunction to the shunt.¹

Conclusions

Early and timely intervention is necessary to treat priapism, which can negatively impact response to treatment and erectile function. Physicians must closely monitor patients for developing erectile dysfunction.

Ethics and patient consent

Written informed consent for the publication of the clinical details and images was obtained from the patient and his family.

Data availability

All data underlying the results are available as part of the article and no additional data sources are required.

References

1. Ali E, Soliman A, De Sanctis V, et al.: Priapism in Patients with Chronic Myeloid Leukemia (CML): A Systematic Review. Acta Biomed. 2021 Jul 1; 92(3): e2021193. PubMed Abstract | Publisher Full Text | Free Full Text
2. Rodgers R, Latif Z, Copland M: How I manage priapism in CML patients. Br. J. Haematol. 2012; 158: 155–164. PubMed Abstract | Publisher Full Text
3. Salonia A, Eardley I, Giuliano F, et al.: European Association of Urology. European Association of Urology guidelines on priapism. Eur. Urol. 2014 Feb; 65(2): 480–489. PubMed Abstract | Publisher Full Text
4. Frazer R, Irvine AE, McMullin MF: Chronic Myeloid Leukaemia in The 21st Century. Ulster Med. J. 2007 Jan; 76(1): 8–17. PubMed Abstract | Free Full Text
6. Goldman JM, Melo JV: Chronic myeloid leukemia--advances in biology and new approaches to treatment. N. Engl. J. Med. 2003 Oct 9; 349(15): 1451–1464. PubMed Abstract | Publisher Full Text
5. O’Brien S, Tefferi A, Valent P: Chronic myelogenous leukemia and myeloproliferative disease. Hematology Am. Soc. Hematol. Educ. Program. 2004; 2004: 146–162. PubMed Abstract | Publisher Full Text
7. Mulhall JP, Honig SC: Priapism: etiology and management. Acad. Emerg. Med. 1996; 3(8): 810–816. Publisher Full Text
8. Kim N, Azadzoi KM, Goldstein I, et al.: A nitric oxide-like factor mediates nonadrenergic-noncholinergic neurogenic relaxation of penile corpus cavernosum smooth muscle. J. Clin. Invest. 1991 Jul; 88(1): 112–118. PubMed Abstract | Publisher Full Text | Free Full Text
9. Burnett AL, Bivalacqua TJ: Priapism: current principles and practice. Urol. Clin. N. Am. 2007 Nov 1; 34(4): 631–642. PubMed Abstract | Publisher Full Text
10. Lugo TG, Pendergast AM, Muller AJ, et al.: Tyrosine kinase activity and transformation potency of bcr-abl oncogene products. Science. 1990; 247: 1079–1082. PubMed Abstract | Publisher Full Text
11. Rowley JD: Letter: a new consistent chromosomal abnormality in chronic myelogenous leukaemia identified by quinacrine fluorescence and Giemsa staining. Nature. 1973; 243: 290–293. PubMed Abstract | Publisher Full Text
12. 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: 111–116. Publisher Full Text
13. Herawati NLP, Dharmayuda TG: Priapism as an initial presentation in Chronic Myeloid. Leukemia (CML): a case report. Intisari Sains Medis. 2021 Dec; 12(1): 334–337. Publisher Full Text
14. Montague DK, Jarow JP, Broderick GA, et al.: Chapter 1: the management of erectile dysfunction: an AUA update. J. Urol. 2005; 174: 230–239. PubMed Abstract | Publisher Full Text
15. Levey HR, Segal RL, Bivalacqua TJ: Management of priapism: an update for clinicians. Ther. Adv. Urol. 2014 Dec; 6(6): 230–244. PubMed Abstract | Publisher Full Text | Free Full Text
16. Pryor J, Hehir M: The management of priapism. Br. J. Urol. 1982; 54: 751–754. Publisher Full Text
17. Burnett A, Pierorazio P: Corporal ‘snake’ maneuver: corporoglanular shunt surgical modification for ischemic priapism. J. Sex. Med. 2009; 6: 1171–1176. PubMed Abstract | Publisher Full Text
18. Höglund M, Sandin F, Simonsson B: Epidemiology of chronic myeloid leukaemia: an update. Ann. Hematol. 2015 Apr; 94(Suppl 2): 241–247. PubMed Abstract | Publisher Full Text
19. Keoghane SR, Sullivan ME, Miller MA: The aetiology, pathogenesis and management of priapism. Br. J. Urol. Int. 2002; 90: 149–154. Publisher Full Text
20. Champion HC, Bivalacqua TJ, Takimoto E, et al.: Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc. Natl. Acad. Sci. U. S. A. 2005; 102: 1661–1666. PubMed Abstract | Publisher Full Text | Free Full Text
21. Bivalacqua TJ, Liu T, Musicki B, et al.: Endothelial nitric oxide synthase keeps erection regulatory function balance in the penis. Eur. Urol. 2007; 51: 1732–1740. PubMed Abstract | Publisher Full Text | Free Full Text
22. Steinhardt GF, Steinhardt E: Priapism in children with leukemia. Urology. 1981; 18: 604–606. Publisher Full Text
23. Emond AM, Holman R, Hayes RJ, et al.: Priapism and Impotence in Homozygous Sickle Cell Disease. Arch. Intern. Med. 1980; 140: 1434–1437. Publisher Full Text
24. Ali EA, Nashwan AJ, Yassin MA: Essential thrombocythemia with (type2) calreticulin presented as stuttering priapism case report and review of literature. Clin. Case Rep. 2021 Jan; 9(1): 399–404. PubMed Abstract | Publisher Full Text | Free Full Text
25. Cherian J, Rao AR, Thwaini A, et al.: Medical and surgical management of priapism. Postgrad. Med. J. 2006; 82: 89–94. PubMed Abstract | Publisher Full Text | Free Full Text
26. Chowdhury ZZ, Al-Asad H, Rahman MH, et al.: Management of priapism with chronic myeloid leukaemia—a rare presentation and our experiences. Haematol. J. Bangladesh. 2020; 3: 39–41. Publisher Full Text
27. Jandial A, Mishra K, Sandal R, et al.: CML patients presenting with priapism: is there any disparity in outcome? J. Clin. Oncol. 2019; 37(15): e18545. Publisher Full Text
28. Kumar M, Garg G, Sharma A, et al.: Comparison of outcomes in malignant vs. non-malignant ischemic priapism: 12-year experience from a tertiary center. Turk. J. Urol. 2019; 45(5): 340–344.
29. Tendulkar AA, Jain PA, Gupta A, et al.: Therapeutic leukocyte reduction for acute and chronic myeloid leukemias: a 4-year experience from an oncology center in India. Asian. J. Transfus. Sci. 2017; 11(2): 156–161. PubMed Abstract | Publisher Full Text | Free Full Text
30. Ekeke O, Omunakwe H, Nwauche C: Chronic myeloid leukaemia presenting as priapism. Int. Surg. 2015; 100: 552–557. PubMed Abstract | Publisher Full Text | Free Full Text
31. Kurosawa H, Tanizawa A, Tono C, et al.: Leukostasis in children and adolescents with chronic myeloid leukemia: Japanese pediatric leukemia/lymphoma study group. Pediatr. Blood Cancer. 2016; 63(3): 406–411. PubMed Abstract | Publisher Full Text
32. Pal DK, Biswal DK, Ghosh B: Outcome and erectile function following treatment of priapism: an institutional experience. Urol Ann. 2016; 8(1): 46–50. Publisher Full Text
33. Nabi G, Dogra PN: Chronic myeloid leukaemia presenting as priapism in children: need for multidisciplinary approach. East Afr. Med. J. 2000; 77(10): 576. PubMed Abstract
34. Gaye O, Thiam NM, Cassell A, et al.: Unusual presentation of priapism associated with acute and chronic myeloid leukemia in two patients: emergency management. Case Rep. Urol. 2020 Aug 12; 2020: 4982432. PubMed Abstract | Publisher Full Text | Free Full Text
35. Dhar J, Dhar J, Chhabra G, et al.: Priapism as a debut presentation of chronic myeloid leukemia. J. Coll. Physicians Surg. Pak. 2019; 29(1): 78–80. Publisher Full Text
36. Qu M, Lu X, Wang L, et al.: Priapism secondary to chronic myeloid leukemia treated by a surgical cavernosa-corpus spongiosum shunt: Case report. Asian J. Urol. 2019; 6(4): 373–376. PubMed Abstract | Publisher Full Text | Free Full Text
37. Becerra-Pedraza LC, Jiménez-Martínez LE, Peña-Morfin I, et al.: Priapism as the initial sign in hematologic disease: Case report and literature review. Int. J. Surg. Case Rep. 2018; 43: 13–17. Publisher Full Text
38. Khan A, Shafiq I, Shah MH, et al.: Chronic myeloid leukaemia presenting as priapism: A case report from Khyber Pakhtunkhwa. J. Pak. Med. Assoc. 2018 Jun 1; 68(6): 942–944. PubMed Abstract
39. Nerli RB, Magdum PV, Hiremath SC, et al.: Priapism - A rare presentation in chronic myeloid leukemia: Case report. Urol. Case Rep. 2016; 4: 8–10. PubMed Abstract | Publisher Full Text | Free Full Text
40. Shaeer OK, Shaeer KZ, Abdelrahman IF, et al.: Priapism as a result of chronic myeloid leukemia: Case report, pathology, and review of the literature. J. Sex. Med. 2015; 12(3): 827–834. PubMed Abstract | Publisher Full Text
41. Farhan S, Anjum F, Al-Qahtani F, et al.: Chronic myeloid leukemia presenting with priapism. J. Leuk. 2014; 3(171): 2.
42. 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(1): 70–73. PubMed Abstract | Publisher Full Text
43. Jameel T, Mehmood K: Priapism - An unusual presentation in Chronic myeloid leukaemia: Case report and review of the literature. Biomedica. 2009; 25: 197–199.
44. Ponniah A, Brown CT, Taylor P, et al.: Priapism secondary to leukemia: effective management with prompt leukapheresis. Int. J. Urol. 2004 Sep; 11(9): 809–810. PubMed Abstract | Publisher Full Text
45. Chang MW, Tang CC, Chang SS, et al.: Priapism—a rare presentation in chronic myeloid leukemia: case report and review of the literature. Chang Gung Med. J. 2003 Apr 1; 26(4): 288–292. PubMed Abstract

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Author details Author details

¹ Urology, Universitas Padjadjaran, Bandung, West Java, 40161, Indonesia

Dahril Dahril
Roles: Conceptualization, Formal Analysis, Methodology, Project Administration, Supervision, Validation, Writing – Review & Editing

Sangaji Ramadhan
Roles: Data Curation, Investigation, Resources, Visualization, Writing – Original Draft Preparation

Competing interests

No competing interests were disclosed.

Grant information

The author(s) declared that no grants were involved in supporting this work.

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Dahril D and Ramadhan S. Case Report: Severe ischemic priapism as the clinical presentation in patient with chronic myeloid leukemia (CML) treated by proximal shunt [version 1; peer review: 1 approved, 1 approved with reservations]. F1000Research 2023, 12:1487 (https://doi.org/10.12688/f1000research.133785.1)

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Reviewer Report 07 May 2024

Muhammad Asykar Palinrungi, Hasanuddin University, Makassar, Indonesia

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https://doi.org/10.5256/f1000research.146798.r264425

This case report is interesting by describing a case of priapism caused by chronic myeloid leukemia (CML) as well as initial and advanced management.
I have several concerns:
Abstract
Introduction: Here the author explains directly the etiology ... Continue reading

This case report is interesting by describing a case of priapism caused by chronic myeloid leukemia (CML) as well as initial and advanced management.
I have several concerns:
Abstract
Introduction: Here the author explains directly the etiology of blood disorders, perhaps the causes of priapism in general can be explained first, then blood disorders.

case report
After carrying out the winter procedure, is an intracavernous alpha adrenergic agent injection not performed? Please provide reasons in the discussion section.
Then proximal shunting is carried out, please provide an explanation of how high it should be done in cases of priapism in the discussion section
Thank You

Is the background of the case’s history and progression described in sufficient detail?

Yes
Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?

Yes
Is sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?

Yes
Is the case presented with sufficient detail to be useful for other practitioners?

Yes

Competing Interests: No competing interests were disclosed.

Reviewer Expertise: urology

I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.

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Reviewer Report 29 Apr 2024

Wulyo Rajabto, Medicine Universitas Indonesia, Jakarta, Indonesia

Approved with Reservations

https://doi.org/10.5256/f1000research.146798.r252626

This case report is very interesting which describes an oncology emergency of how we treat priapism caused by chronic myeloid leukemia (CML).
The physical examination of patients with CML usually shows splenomegaly, however, this case report does not show ... Continue reading

This case report is very interesting which describes an oncology emergency of how we treat priapism caused by chronic myeloid leukemia (CML).
The physical examination of patients with CML usually shows splenomegaly, however, this case report does not show the spleen size. The laboratory test of patient with CML usually reveals mild anemia, however in this case report the authors did not show the Hb level, severe leukocytosis (just like in this patient) with differential count 87/87/3/3/5x103/uL, however this differential count is not correct, I think. There are many identifiable details about the patient's identity in the ultrasound images, please hide the name of the patient and the name of the hospital. I believe that the initial treatment of the patient with priapism is the aspiration of blood of the corpus penis, however the authors performed a winter procedure directly. The discussion shows a great detail about the diagnosis, the treatment of CML, and the treatment of urology procedure how to combat the priapism.

Overall, I believe the case presented with sufficient detail to be useful for other practitioners.

Is the background of the case’s history and progression described in sufficient detail?

Yes
Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?

Partly
Is sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?

Yes
Is the case presented with sufficient detail to be useful for other practitioners?

Yes

Competing Interests: No competing interests were disclosed.

Reviewer Expertise: CML: epidemiology, diagnosis, and treatment.

I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above.

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VERSION 1 PUBLISHED 20 Nov 2023

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Wulyo Rajabto, Medicine Universitas Indonesia, Jakarta, Indonesia
Muhammad Asykar Palinrungi, Hasanuddin University, Makassar, Indonesia

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Reviewer Report

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07 May 2024 | for Version 1

Muhammad Asykar Palinrungi, Hasanuddin University, Makassar, Indonesia

2 Views Cite this report Responses(0)

Approved

This case report is interesting by describing a case of priapism caused by chronic myeloid leukemia (CML) as well as initial and advanced management.
I have several concerns:
Abstract
Introduction: Here the author explains directly the etiology of blood disorders, perhaps the causes of priapism in general can be explained first, then blood disorders.

case report
After carrying out the winter procedure, is an intracavernous alpha adrenergic agent injection not performed? Please provide reasons in the discussion section.
Then proximal shunting is carried out, please provide an explanation of how high it should be done in cases of priapism in the discussion section
Thank You

Is the background of the case’s history and progression described in sufficient detail?

Yes
Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?

Yes
Is sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?

Yes
Is the case presented with sufficient detail to be useful for other practitioners?

Yes

Competing Interests

No competing interests were disclosed.

Reviewer Expertise

urology

I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.

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Reviewer Report

7 Views

29 Apr 2024 | for Version 1

Wulyo Rajabto, Medicine Universitas Indonesia, Jakarta, Indonesia

7 Views Cite this report Responses(0)

Approved With Reservations

This case report is very interesting which describes an oncology emergency of how we treat priapism caused by chronic myeloid leukemia (CML).
The physical examination of patients with CML usually shows splenomegaly, however, this case report does not show the spleen size. The laboratory test of patient with CML usually reveals mild anemia, however in this case report the authors did not show the Hb level, severe leukocytosis (just like in this patient) with differential count 87/87/3/3/5x103/uL, however this differential count is not correct, I think. There are many identifiable details about the patient's identity in the ultrasound images, please hide the name of the patient and the name of the hospital. I believe that the initial treatment of the patient with priapism is the aspiration of blood of the corpus penis, however the authors performed a winter procedure directly. The discussion shows a great detail about the diagnosis, the treatment of CML, and the treatment of urology procedure how to combat the priapism.

Overall, I believe the case presented with sufficient detail to be useful for other practitioners.

Is the background of the case’s history and progression described in sufficient detail?

Yes
Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?

Partly
Is sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?

Yes
Is the case presented with sufficient detail to be useful for other practitioners?

Yes

Competing Interests

No competing interests were disclosed.

Reviewer Expertise

CML: epidemiology, diagnosis, and treatment.

I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above.

Respond to this report

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[1] 1. Ali E, Soliman A, De Sanctis V, et al.: Priapism in Patients with Chronic Myeloid Leukemia (CML): A Systematic Review. Acta Biomed. 2021 Jul 1; 92(3): e2021193. PubMed Abstract | Publisher Full Text | Free Full Text

[2] 2. Rodgers R, Latif Z, Copland M: How I manage priapism in CML patients. Br. J. Haematol. 2012; 158: 155–164. PubMed Abstract | Publisher Full Text

[3] 3. Salonia A, Eardley I, Giuliano F, et al.: European Association of Urology. European Association of Urology guidelines on priapism. Eur. Urol. 2014 Feb; 65(2): 480–489. PubMed Abstract | Publisher Full Text

[4] 4. Frazer R, Irvine AE, McMullin MF: Chronic Myeloid Leukaemia in The 21st Century. Ulster Med. J. 2007 Jan; 76(1): 8–17. PubMed Abstract | Free Full Text

[5] 6. Goldman JM, Melo JV: Chronic myeloid leukemia--advances in biology and new approaches to treatment. N. Engl. J. Med. 2003 Oct 9; 349(15): 1451–1464. PubMed Abstract | Publisher Full Text

[6] 5. O’Brien S, Tefferi A, Valent P: Chronic myelogenous leukemia and myeloproliferative disease. Hematology Am. Soc. Hematol. Educ. Program. 2004; 2004: 146–162. PubMed Abstract | Publisher Full Text

[7] 7. Mulhall JP, Honig SC: Priapism: etiology and management. Acad. Emerg. Med. 1996; 3(8): 810–816. Publisher Full Text

[8] 8. Kim N, Azadzoi KM, Goldstein I, et al.: A nitric oxide-like factor mediates nonadrenergic-noncholinergic neurogenic relaxation of penile corpus cavernosum smooth muscle. J. Clin. Invest. 1991 Jul; 88(1): 112–118. PubMed Abstract | Publisher Full Text | Free Full Text

[9] 9. Burnett AL, Bivalacqua TJ: Priapism: current principles and practice. Urol. Clin. N. Am. 2007 Nov 1; 34(4): 631–642. PubMed Abstract | Publisher Full Text

[10] 10. Lugo TG, Pendergast AM, Muller AJ, et al.: Tyrosine kinase activity and transformation potency of bcr-abl oncogene products. Science. 1990; 247: 1079–1082. PubMed Abstract | Publisher Full Text

[11] 11. Rowley JD: Letter: a new consistent chromosomal abnormality in chronic myelogenous leukaemia identified by quinacrine fluorescence and Giemsa staining. Nature. 1973; 243: 290–293. PubMed Abstract | Publisher Full Text

[12] 12. 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: 111–116. Publisher Full Text

[13] 13. Herawati NLP, Dharmayuda TG: Priapism as an initial presentation in Chronic Myeloid. Leukemia (CML): a case report. Intisari Sains Medis. 2021 Dec; 12(1): 334–337. Publisher Full Text

[14] 14. Montague DK, Jarow JP, Broderick GA, et al.: Chapter 1: the management of erectile dysfunction: an AUA update. J. Urol. 2005; 174: 230–239. PubMed Abstract | Publisher Full Text

[15] 15. Levey HR, Segal RL, Bivalacqua TJ: Management of priapism: an update for clinicians. Ther. Adv. Urol. 2014 Dec; 6(6): 230–244. PubMed Abstract | Publisher Full Text | Free Full Text

[16] 16. Pryor J, Hehir M: The management of priapism. Br. J. Urol. 1982; 54: 751–754. Publisher Full Text

[17] 17. Burnett A, Pierorazio P: Corporal ‘snake’ maneuver: corporoglanular shunt surgical modification for ischemic priapism. J. Sex. Med. 2009; 6: 1171–1176. PubMed Abstract | Publisher Full Text

[18] 18. Höglund M, Sandin F, Simonsson B: Epidemiology of chronic myeloid leukaemia: an update. Ann. Hematol. 2015 Apr; 94(Suppl 2): 241–247. PubMed Abstract | Publisher Full Text

[19] 19. Keoghane SR, Sullivan ME, Miller MA: The aetiology, pathogenesis and management of priapism. Br. J. Urol. Int. 2002; 90: 149–154. Publisher Full Text

[20] 20. Champion HC, Bivalacqua TJ, Takimoto E, et al.: Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc. Natl. Acad. Sci. U. S. A. 2005; 102: 1661–1666. PubMed Abstract | Publisher Full Text | Free Full Text

[21] 21. Bivalacqua TJ, Liu T, Musicki B, et al.: Endothelial nitric oxide synthase keeps erection regulatory function balance in the penis. Eur. Urol. 2007; 51: 1732–1740. PubMed Abstract | Publisher Full Text | Free Full Text

[22] 22. Steinhardt GF, Steinhardt E: Priapism in children with leukemia. Urology. 1981; 18: 604–606. Publisher Full Text

[23] 23. Emond AM, Holman R, Hayes RJ, et al.: Priapism and Impotence in Homozygous Sickle Cell Disease. Arch. Intern. Med. 1980; 140: 1434–1437. Publisher Full Text

[24] 24. Ali EA, Nashwan AJ, Yassin MA: Essential thrombocythemia with (type2) calreticulin presented as stuttering priapism case report and review of literature. Clin. Case Rep. 2021 Jan; 9(1): 399–404. PubMed Abstract | Publisher Full Text | Free Full Text

[25] 25. Cherian J, Rao AR, Thwaini A, et al.: Medical and surgical management of priapism. Postgrad. Med. J. 2006; 82: 89–94. PubMed Abstract | Publisher Full Text | Free Full Text

[26] 26. Chowdhury ZZ, Al-Asad H, Rahman MH, et al.: Management of priapism with chronic myeloid leukaemia—a rare presentation and our experiences. Haematol. J. Bangladesh. 2020; 3: 39–41. Publisher Full Text

[27] 27. Jandial A, Mishra K, Sandal R, et al.: CML patients presenting with priapism: is there any disparity in outcome? J. Clin. Oncol. 2019; 37(15): e18545. Publisher Full Text

[28] 28. Kumar M, Garg G, Sharma A, et al.: Comparison of outcomes in malignant vs. non-malignant ischemic priapism: 12-year experience from a tertiary center. Turk. J. Urol. 2019; 45(5): 340–344.

[29] 29. Tendulkar AA, Jain PA, Gupta A, et al.: Therapeutic leukocyte reduction for acute and chronic myeloid leukemias: a 4-year experience from an oncology center in India. Asian. J. Transfus. Sci. 2017; 11(2): 156–161. PubMed Abstract | Publisher Full Text | Free Full Text

[30] 30. Ekeke O, Omunakwe H, Nwauche C: Chronic myeloid leukaemia presenting as priapism. Int. Surg. 2015; 100: 552–557. PubMed Abstract | Publisher Full Text | Free Full Text

[31] 31. Kurosawa H, Tanizawa A, Tono C, et al.: Leukostasis in children and adolescents with chronic myeloid leukemia: Japanese pediatric leukemia/lymphoma study group. Pediatr. Blood Cancer. 2016; 63(3): 406–411. PubMed Abstract | Publisher Full Text

[32] 32. Pal DK, Biswal DK, Ghosh B: Outcome and erectile function following treatment of priapism: an institutional experience. Urol Ann. 2016; 8(1): 46–50. Publisher Full Text

[33] 33. Nabi G, Dogra PN: Chronic myeloid leukaemia presenting as priapism in children: need for multidisciplinary approach. East Afr. Med. J. 2000; 77(10): 576. PubMed Abstract

[34] 34. Gaye O, Thiam NM, Cassell A, et al.: Unusual presentation of priapism associated with acute and chronic myeloid leukemia in two patients: emergency management. Case Rep. Urol. 2020 Aug 12; 2020: 4982432. PubMed Abstract | Publisher Full Text | Free Full Text

[35] 35. Dhar J, Dhar J, Chhabra G, et al.: Priapism as a debut presentation of chronic myeloid leukemia. J. Coll. Physicians Surg. Pak. 2019; 29(1): 78–80. Publisher Full Text

[36] 36. Qu M, Lu X, Wang L, et al.: Priapism secondary to chronic myeloid leukemia treated by a surgical cavernosa-corpus spongiosum shunt: Case report. Asian J. Urol. 2019; 6(4): 373–376. PubMed Abstract | Publisher Full Text | Free Full Text

[37] 37. Becerra-Pedraza LC, Jiménez-Martínez LE, Peña-Morfin I, et al.: Priapism as the initial sign in hematologic disease: Case report and literature review. Int. J. Surg. Case Rep. 2018; 43: 13–17. Publisher Full Text

[38] 38. Khan A, Shafiq I, Shah MH, et al.: Chronic myeloid leukaemia presenting as priapism: A case report from Khyber Pakhtunkhwa. J. Pak. Med. Assoc. 2018 Jun 1; 68(6): 942–944. PubMed Abstract

[39] 39. Nerli RB, Magdum PV, Hiremath SC, et al.: Priapism - A rare presentation in chronic myeloid leukemia: Case report. Urol. Case Rep. 2016; 4: 8–10. PubMed Abstract | Publisher Full Text | Free Full Text

[40] 40. Shaeer OK, Shaeer KZ, Abdelrahman IF, et al.: Priapism as a result of chronic myeloid leukemia: Case report, pathology, and review of the literature. J. Sex. Med. 2015; 12(3): 827–834. PubMed Abstract | Publisher Full Text

[41] 41. Farhan S, Anjum F, Al-Qahtani F, et al.: Chronic myeloid leukemia presenting with priapism. J. Leuk. 2014; 3(171): 2.

[42] 42. 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(1): 70–73. PubMed Abstract | Publisher Full Text

[43] 43. Jameel T, Mehmood K: Priapism - An unusual presentation in Chronic myeloid leukaemia: Case report and review of the literature. Biomedica. 2009; 25: 197–199.

[44] 44. Ponniah A, Brown CT, Taylor P, et al.: Priapism secondary to leukemia: effective management with prompt leukapheresis. Int. J. Urol. 2004 Sep; 11(9): 809–810. PubMed Abstract | Publisher Full Text

[45] 45. Chang MW, Tang CC, Chang SS, et al.: Priapism—a rare presentation in chronic myeloid leukemia: case report and review of the literature. Chang Gung Med. J. 2003 Apr 1; 26(4): 288–292. PubMed Abstract

Case Report: Severe ischemic priapism as the clinical presentation in patient with chronic myeloid leukemia (CML) treated by proximal shunt

Abstract

Keywords

Introduction

Case report

Figure 1. The genitalia showed a painful erection (three days).

Figure 2. Peripheral blood smear results suggesting a hematologic malignancy.

Figure 3. Ultrasonography (USG) and Doppler Ultrasound (US) results showing low-flow priapism with penile edema.

Figure 4. The patient after Winter’s procedure (distal shunt).

Figure 5. The patient then underwent surgery through the proximal shunting.

Figure 6. The patient after proximal shunting.

Discussion

Chronic myeloid leukaemia (CML)

Table 1. Overview of case reports and reviews.

Priapism

Classification of priapism

Pathophysiology of priapism

Table 2. Instances of priapism and leukemia have been reported in the past two decades.

Surgical management of priapism

Priapism in CML patients

Conclusions

Ethics and patient consent

Data availability

References

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