Leukaemia-associated priapism in children (LAPC): reviewing clinical outcomes and management strategies
Abhijit Shah1, Surag KR1, Anupam Choudhary1, Kasi Viswanath1, Krishnakanth AVB1, Chaitanya Krishna1, Padmaraj Hegde1, Gayathri S2 and Swathi PM3
1Department of Urology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India
2Department of Clinical Hematology, Cellular therapy and Bone marrow transplant, Amrita Institute of Medica Sciences, Kochi, Kerala, India
3Department of Pediatric Hemato-Oncology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India
Abstract
Background: Priapism is a prolonged penile erection for more than 4 hours unrelated to sexual stimulation. Rarely, it is the first clinical sign of an underlying haematological malignancy. A similar presentation is noted in childhood leukaemias. Although rare, it is known to occur and, if not managed early, can have poor long-term outcomes in terms of erectile function and psychosexual growth. We present a scoping review of leukaemia-associated priapism in children (LAPC).
Methodology: We researched literature using PubMed, Google Scholar, Embase, Scopus and Cochrane databases from January 1990 to 2024. Applicable search limiters were applied, and grey literature was excluded.
Results: A total of 31 articles were finally included in the review, from which 51 cases of LAPC were isolated and studied. The average age was 11.5 years, with chronic myeloid leukaemia (CML) being the most common malignancy (68.9%), and more than 71% of cases of CML with priapism were detected in the chronic phase. Twenty cases (39.2%) were managed with corporal lavage and sympathomimetic injections at the initial onset, with the rest managed with cytoreductive measures initially. Follow-up data revealed the death of three children, whereas, of those that survived, fourteen had preserved erectile functions after a variable period of time.
Conclusion: Priapism in children warrants a thorough physical examination focusing on organomegaly and a complete hemogram. Initial management should be two-pronged with a priapism-directed corporal-lavage approach and cytoreductive measures for better long-term outcomes.
Keywords: leukaemia, priapism, child, outcomes assessment, healthcare, management
Correspondence to: Swathi PM
Email: swathi.pm@manipal.edu
Published: 27/02/2025
Received: 18/09/2024
Publication costs for this article were supported by ecancer (UK Charity number 1176307).
Copyright: © the authors; licensee ecancermedicalscience. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Introduction
Priapism refers to the prolonged and persistent erection of the penis, hours beyond (>4 hours) or unrelated to sexual stimulation [1,2]. This persistent erection is due to persistently engorged corpus cavernosum secondary to a vascular disturbance that typically controls penile rigidity. Sickle cell disease is the most common cause of priapism in children (65%), whereas leukaemia-associated priapism in children (LAPC) incidence rates are 10% [3]. Data on LAPC are sparse, and so are the outcomes of its management.
The present review is undertaken to (a) assess the characteristics and types of leukaemia and their role in LAPC, (b) describe the management options available and (c) investigate the outcomes.
Methodology
We researched English literature from January 1990 to 2024 (PubMed, Google Scholar, Cochrane, Scopus and Embase) for original articles, reviews, case series and case reports for search terms ‘Priapism’ and (‘Leukaemia’ or ‘Lymphoma’) and (‘childhood’ or ‘all children (birth-18 years)’), excluding terms ‘sickle cell’, ‘female’ and ‘Adult’. We also excluded grey literature data from this review. Thirty-one studies were finally included in our review after excluding duplicates, including those with no full text, and applying our limiters (Figure 1). Our study was registered with PROSPERO ‘CRD42024519022’, and PRISMA-ScR reporting guidelines [4] were adhered to.
Results
We reviewed a total of 51 cases in 31 different studies. The overall data on LAPC are quite scarce, and this is noted in numerous case reports (28 out of 31 studies). The remaining three studies are case series (≥3 cases), which include a population-based registry study on chronic myeloid leukaemia (CML) and another paper studying the impact of leukapheresis in acute myeloid leukaemia (AML) [5–7]. One case report mentions two cases, one of which was of an adult. Hence, only the paediatric case has been included [8].
Figure 1. The PRISMA flow diagram detailing the search of articles involving children with leukaemia-associated priapism.
The median age in our review was 12 years (3–17 years), and the median duration, in 37.3% of cases after the onset of priapism to presentation in the hospital, was 24 hours (n = 19). However, various reports mention recurring or stuttering priapism occurring a few days or a few weeks before the presentation, with no mention of the exact time of presentation from the latest episode of priapism [5, 9, 10]
The search showed the most frequent disorder being CML, 68.9% (n = 35). The chronic phase of CML was the most common phase of onset of priapism and presentation to the clinician (71.4%), followed by blast crisis and accelerated phase (11.4% and 8.6%, respectively). The four remaining reports did not mention the blast percentages or the phase of CML. The median age of the CML cohort was 14 years (4–17 years) Of the 35 cases, 32 cases had palpable splenomegaly, and three reports failed to mention the examination findings. The median white blood cell (WBC) count was 450,000/µL (135,000/µL–899,000/µL). Table 1 reviews chronic leukaemia cases and their characteristics, and Table 2 reviews the priapism-directed management and eventual outcomes. Apart from imatinib, hydroxyurea, allopurinol and steroids were recorded medications in these case reports. Thirty-seven percent of cases (n = 13) report using corporal lavage with saline or phenylephrine injections and adequate hydration as the initial management step. Surgical intervention in the form of a distal shunt (Winter and Ebbehoj) was performed in six cases, of which one needed revision to a proximal shunt procedure for eventual detumescence [11]. One case documented bilateral internal iliac artery embolization [12]. Very few of these studies mentioned the eventual follow-up outcomes. Of those, three cases of erectile dysfunction [12–14] were mentioned, along with one death secondary to pneumonitis post-stem cell transplant [5].
Of the acute leukaemias (Table 3) associated with priapism, the median age was younger (9.3 years and 3–16 years), and the most common subtype was B cell acute lymphoblastic leukaemia (ALL) followed by T cell ALL and AML. Of the 16 cases of acute leukaemias, 11.8% (n = 6) were B-ALL cases, 9.8% (n = 5) cases were T-ALL, 5.9% (n = 3) AML and 2 cases were ALL with subtype not mentioned. The median WBC in acute leukaemia cases noted was 423,500/µL (27,300/µL–693,000/µL). Acute leukaemias were overall associated with a rapid progression of the underlying disease with an earlier presentation (<24 hours in 43.8% of acute cases, n = 7) and two reports of death due to central nervous system (CNS) leukaemia [15] and septic shock [16]. From the available data, 7 cases (43.8%) of acute leukaemias were in remission. However, the overall long-term erectile function outcomes in this cohort are not available (except in one case where the erectile function was preserved [6]).
Discussion
Etiopathogenesis
Hinman [17] first reported the ischemic nature of priapism based on aspiration of deoxygenated blood due to increased viscosity, congestion and reduced flow. Eponymous with the Greek–Roman mythological figure Priapus, son of Aphrodite, priapism was first described in the literature in 1616 by Petraens. It was almost 260 years later; this condition was first recorded in a child [18, 19]
Priapism is subdivided into three types: high-flow/non-ischemic/arterial type, low-flow/ischemic type and stuttering/recurring type (common in sickle cell disease). It is the low-flow/ischemic type that is painful, may result in irreversible damage through fibrosis if left untreated for 24–36 hours and has been strongly implicated with leading to impotence in 35%–90% of men [20–24]. It becomes essential to differentiate the clinical type of priapism to prevent long-term, irreversible effects [1, 25–27].
Priapism in leukaemia is hypothesised to occur via different mechanisms [28–30]. The most likely mechanism could occur due to venous obstruction secondary to microemboli/thrombi and increased microviscosity due to increased circulating blood cells (‘symptomatic hyperleukocytosis’, also known as ‘leukostasis’). Hyperleukocytosis is defined as a WBC greater than 1,00,000/µL in patients affected by leukaemias. The incidence of hyperleukocytosis ranges from 5% to 13% in AML and from 10% to 30% in ALL [31]. However, symptomatic hyperleukocytosis is higher in AML than in ALL [32] and also typically occurs with much higher WBC counts in the case of ALL than in patients with AML. The reason is that myeloid blasts are larger and more rigid than lymphoid blasts, and myeloid blasts secrete cytokines, which upregulate endothelial cell adhesion, whereas in chronic leukaemia such as CML, WBCs are usually segmented neutrophils, metamyelocytes and myelocytes, which are smaller and more deformable. Hence, symptomatic hyperleukocytosis is very rare in this patient population and is mostly seen in the accelerated phase or blast crisis or the overall leukocyte counts are extremely high for the symptoms of LAPC to occur [29, 30, 33, 34].
Table 1. Characteristics of LAPC with CML.
Table 2. Management and outcomes of LAPC with CML.
Table 3. Characteristics, management and outcome of LAPC with acute leukaemia.
Management and long-term outcomes
Priapism is a urological emergency that needs to be addressed early to prevent permanent risk of erectile dysfunction, which is predicted to be >90% if it lasts for more than 24 hours [41]. Children with underlying leukaemia need to be started on disease-directed therapy at the earliest, be it chemotherapy, tyrosine kinase inhibitor or cytoreductive measures with hydration, hydroxyurea and leukapheresis when warranted. However, these measures should not defer the emergency treatment of priapism itself.
Figure 2 depicts a proposed management algorithm for patients presenting with LAPC. The initial management is crucial as it dictates the erectile outcomes and long-term prognosis of these patients. In our study, 20 cases (39.2%) were reportedly subjected to corporal lavage in addition to disease-directed measures. The rest were managed with cytoreductive measures supplemented with adequate IV hydration and analgesia.
Systemic interventions, such as cytoreductive measures with hydroxyurea, allopurinol and steroids, have been shown to reduce the white cell count [5, 42]. In cases where an acute control of leucocyte count is warranted or the cytoreductive measures fail to establish a reduced leucocyte count, leukapheresis or exchange transfusion methods are commonly employed [5, 43, 44]. Seventeen patients (33%) underwent leukapheresis, of which 12 were noted in CML cases. Suttorp et al [5], Ponniah et al [43] and Veljković et al [45] all demonstrate its usefulness for CML cases associated with priapism.
Certain adjunct treatments noted in our review worth revisiting were bilateral internal iliac artery embolization with urokinase [12] and epidural analgesia [37]. If a paediatric anaesthetist is present, low-dose ketamine has been suggested to resolve priapism in some cases and may help to relieve pain during corporal lavage and shunt procedures in these children [46–50]
Figure 2. Algorithm for management of LAPC.
From a surgical standpoint, a urologist must be aware of various types of shunt procedures that may be performed in cases of unresolved priapism. Distal shunts, such as the Winter, Ebbehoj and T-Shunt, are easier to create. Seideman and Gitlin [51] describe the successful use of a T-Shunt in refractory ischemic priapism in a paediatric patient. In this review, we report a similar use of distal shunts in refractory priapism [5, 14, 52–55]. Thakur et al [11] report failure of distal shunt, which was later converted to a proximal shunt with success.
Fifteen cases (29.4%) from the entire cohort reported a preserved erectile function on follow-up. Follow-up data are as sparse as the original literature on LAPC. However, it is of utmost importance that on the initial presentation itself, the patient’s family must be made aware of the possible loss of erectile function in the future, despite the clinician’s best efforts. Teenagers and their parents often do not discuss sexual health in boys [26, 42]. This was also witnessed in a cohort of boys with sickle cell disease with priapism, who reported embarrassment to report their complaints to their parents, even in the emergency room when it became a medical emergency [27].
There are definite limitations present in this review, the first of which is the prevalence of only case reports, as this condition is rare. This gives rise to varied management methods, which need streamlining, as we attempted in our study. Follow-up data on many children in terms of erectile status remain unknown. A multi-institutional collaboration with their respective registries may further help define a protocol to manage LAPC and help to trace and contact the patient or next of kin and provide follow-up data.
Conclusion
Though rare, priapism may be the only presenting symptom of haematological malignancies in children. Thus, it is essential to examine for organomegaly and do a complete hemogram with peripheral smear examination in all children presenting with priapism. Considering ischemic priapism, a urological emergency and the potential risk of permanent damage later in life, managing solely with systemic anti-leukaemia therapies (chemotherapy and/or leucopheresis) may not be sufficient for early resolution. Concurrent cavernosal management with corporal lavage and injectable sympathomimetics may help resolve the acute condition and prevent long-term erectile sequelae and psychosocial problems.
Conflicts of interest
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
Funding
This research did not receive any specific grant funding agencies in the public, commercial or not-for-profit sectors.
References
1. Bivalacqua TJ, Allen BK, and Brock GB, et al (2022) The diagnosis and management of priapism: an AUA/SMSNA guideline (2022) executive summary J Urol 208(1) 43–52 https://doi.org/10.1097/JU.0000000000002767 PMID: 35536142
2. Salonia A, Bettocchi C, and Boeri L, et al (2021) European Association of Urology guidelines on sexual and reproductive health-2021 update: male sexual dysfunction Eur Urol 80 333–357 https://doi.org/10.1016/j.eururo.2021.06.007 PMID: 34183196
3. Burnett AL and Bivalacqua TJ (2007) Priapism: current principles and practice Urol Clin North Am 34(4) 631–642 https://doi.org/10.1016/j.ucl.2007.08.006 PMID: 17983902
4. Tricco AC, Lillie E, and Zarin W, et al (2018) PRISMA extension for scoping reviews (PRISMA-ScR): checklist and explanation Ann Intern Med 169 467–473 https://doi.org/10.7326/M18-0850 PMID: 30178033
5. Suttorp M, Sembill S, and Kalwak K, et al (2023) Priapism at diagnosis of pediatric chronic myeloid leukemia: data derived from a large cohort of children and teenagers and a narrative review on priapism management J Clin Med 12(14) 4776 https://doi.org/10.3390/jcm12144776 PMID: 37510891 PMCID: 10380995
6. Castagnetti M, Sainati L, and Giona F, et al (2008) Conservative management of priapism secondary to leukemia Pediatr Blood Cancer 51 420–423 https://doi.org/10.1002/pbc.21628 PMID: 18506758
7. Bruserud O, Liseth K, and Stamnesfet S, et al (2013) Hyperleukocytosis and leukocytapheresis in acute leukaemias: experience from a single centre and review of the literature of leukocytapheresis in acute myeloid leukaemia Transfus Med 23 397–406 https://doi.org/10.1111/tme.12067 PMID: 23919332
8. Gaye O, Thiam NM, and Cassell A, et al (2020) Unusual presentation of priapism associated with acute and chronic myeloid leukemia in two patients: emergency management Case Rep Urol 13 1–5
9. Salou S, Feige U, and Antoniou E, et al (2022) Priapism from myeloid sarcoma of the sacral canal in a 6-year-old patient Pediatr Hematol Oncol 39 83–88 https://doi.org/10.1080/08880018.2021.1928803
10. Gumruk F, Hiçsónmez G, and Özsoylu S, et al (1991) High-dose methylprednisolone for the treatment of priapism in childhood leukemia Pediatr Hematol Oncol 8(4) 367–368 https://doi.org/10.3109/08880019109028811 PMID: 1782116
11. Thakur P, Verma V, and Fotedar V, et al (2019) Priapism in a pediatric chronic myeloid leukaemia patient: unusual presentation of a rare disease in children Clin Cancer Investig J 8(2) 76–78
12. Murayama K, Shibuya A, and Ishii S, et al (2001) Embolization of the bilateral internal pudendal arteries for intractable priapism in a child with chronic myelogenous leukemia Rinsho Ketsueki 42(11) 1117–1121
13. Sachdeva P, Kalra M, and Thatikonda KB, et al (2021) Stuttering priapism in a teenage boy: lesson to be learnt J Pediatr Hematol Oncol 43(8) 1118–1119 https://doi.org/10.1097/MPH.0000000000001998
14. Romero-Guerra AL, Salas-Cosio MJ, and Bautista-Martínez BA, et al (2024) Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report. Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso Arch Argent Pediatr 122(2) e202310068
15. Gupta G, Kumar D, and Trivedi M (2020) Acute lymphoblastic leukemia in a child presenting primarily with priapism J Indian Assoc Pediatr Surg 25(1) 52–54 https://doi.org/10.4103/jiaps.JIAPS_214_18 PMID: 31896902 PMCID: 6910052
16. Yildiz I, Ozşahin H, and Ozbek S (1991) Priapism in a child with acute lymphoblastic leukemia Acta Paediatr Scand 80(5) 553–554 https://doi.org/10.1111/j.1651-2227.1991.tb11904.x PMID: 1872181
17. Hinman F (1960) Priapism; reasons for failure of therapy J Urol 83(4) 420–428 https://doi.org/10.1016/S0022-5347(17)65731-8 PMID: 14401886
18. Cherian J, Rao AR, and Thwaini A, et al (2006) Medical and surgical management of priapism Postgrad Med J 82(964) 89–94 https://doi.org/10.1136/pgmj.2005.037291 PMID: 16461470 PMCID: 2596691
19. Dust N, Daboval T, and Guerra L (2011) Evaluation and management of priapism in a newborn: A case report and review of the literature Paediatr Child Health 16(1) 6–8 https://doi.org/10.1093/pch/16.1.e6
20. Fowler JE Jr, Koshy M, and Strub M, et al (1991) Priapism associated with the sickle cell hemoglobinopathies: prevalence, natural history and sequelae J Urol 145(1) 65–68 https://doi.org/10.1016/S0022-5347(17)38248-4 PMID: 1984102
21. Dekalo S, Stern N, and Broderick GA, et al (2022) Priapism or prolonged erection: is 4–6 hours of cavernous ischemia the time point of irreversible tissue injury? Sex Med Rev 10(4) 660–668 https://doi.org/10.1016/j.sxmr.2022.06.007 PMID: 36028433
22. Vilke GM, Harrigan RA, and Ufberg JW, et al (2004) Emergency evaluation and treatment of priapism J Emerg Med 26(3) 325–329 https://doi.org/10.1016/j.jemermed.2003.12.011 PMID: 15028333
23. Morano SG, Latagliata R, and Carmosino I, et al (2000) Treatment of long-lasting priapism in chronic myeloid leukemia at onset Ann Hematol 79(11) 644–645 https://doi.org/10.1007/s002770000199 PMID: 11131926
24. Pryor J, Akkus E, and Alter G, et al (2004) Priapism J Sex Med 1(1) 116–120 https://doi.org/10.1111/j.1743-6109.2004.10117.x
25. Idris IM, Abba A, and Galadanci JA, et al (2020) Men with sickle cell disease experience greater sexual dysfunction when compared with men without sickle cell disease Blood Adv 4(14) 3277–3283 https://doi.org/10.1182/bloodadvances.2020002062 PMID: 32702096 PMCID: 7391159
26. Donaldson JF, Rees RW, and Steinbrecher HA (2014) Priapism in children: a comprehensive review and clinical guideline J Pediatr Urol 10 11–24 https://doi.org/10.1016/j.jpurol.2013.07.024
27. Addis G, Spector R, and Shaw E, et al (2007) The physical, social and psychological impact of priapism on adult males with sickle cell disorder Chronic Illn 3(2) 145–154 https://doi.org/10.1177/1742395307081505 PMID: 18083669
28. Mulhall JP and Honig SC (1996) Priapism: etiology and management Acad Emerg Med 3(8) 810–816 https://doi.org/10.1111/j.1553-2712.1996.tb03520.x PMID: 8853679
29. Toepfner N, Herold C, and Otto O, et al (2018) Detection of human disease conditions by single-cell morpho-rheological phenotyping of blood Elife 7 e29213 https://doi.org/10.7554/eLife.29213 PMID: 29331015 PMCID: 5790376
30. Lichtman MA (1973) Rheology of leukocytes, leukocyte suspensions, and blood in leukemia. Possible relationship to clinical manifestations J Clin Invest 52(2) 350–358 https://doi.org/10.1172/JCI107191 PMID: 4509637 PMCID: 302264
31. Inaba H, Fan Y, and Pounds S, et al (2008) Clinical and biologic features and treatment outcome of children with newly diagnosed acute myeloid leukemia and hyperleukocytosis Cancer 113(3) 522–529 https://doi.org/10.1002/cncr.23581 PMID: 18484648
32. Porcu P, Cripe LD, and Ng EW, et al (2000) Hyperleukocytic leukemias and leukostasis: a review of pathophysiology, clinical presentation and management Leuk Lymphoma 39 1–18 https://doi.org/10.3109/10428190009053534 PMID: 10975379
33. Lam WA, Rosenbluth MJ, and Fletcher DA (2008) Increased leukaemia cell stiffness is associated with symptoms of leucostasis in paediatric acute lymphoblastic leukaemia Br J Haematol 142(3) 497–501 https://doi.org/10.1111/j.1365-2141.2008.07219.x PMID: 18544083 PMCID: 7899135
34. Bashant KR, Toepfner N, and Day CJ, et al (2020) The mechanics of myeloid cells Biol Cell 112(4) 103–112 https://doi.org/10.1111/boc.201900084 PMID: 31916263
35. Chaux A, Amin M, and Cubilla AL, et al (2011) Metastatic tumors to the penis Int J Surg Pathol 19(5) 597–606 https://doi.org/10.1177/1066896909350468
36. More S, Kotru M, and Sikka M, et al (2020) Priapism in childhood B-cell acute lymphoblastic leukemia: a rare presentation Indian J Hematol Blood Transfus 36 215–216 https://doi.org/10.1007/s12288-019-01186-7 PMID: 32174697 PMCID: 7042465
37. Gokce A, Gul D, and Orhan MF, et al (2021) Epidural blockade for alternative management of priapism: a case report of child with T-cell acute lymphoblastic leukemia Urology 156 e121–123 https://doi.org/10.1016/j.urology.2021.06.017 PMID: 34186129
38. Choo-Kang Leer, Jones DM, and Fehr JJ, et al (1999) Cerebral edema and priapism in an adolescent with acute lymphoblastic leukemia Pediatr Emerg Care 15(2) 110–112 https://doi.org/10.1097/00006565-199904000-00009
39. Mustafa M, Hanafy E, and Riyad S, et al (2024) Priapism as an unusual symptom of T-cell acute lymphoblastic leukemia in a pediatric case Cureus 16(2) e54331. PMID: 38500890 PMCID: 10944801
40. Olcay L, Aribaş BK, and Gökçe M (2009) A patient with acute myeloblastic leukemia who presented with conus Medullaris Syndrome and review of the literature J Pediatr Hematol Oncol 31(6) 440–447 https://doi.org/10.1097/MPH.0b013e31819ed24b PMID: 19648794
41. Ali E, Soliman A, and De Sanctis V, et al (2021) Priapism in patients with chronic myeloid leukemia (CML): a systematic review Acta Biomed 92(3) e2021193 PMID: 34212918 PMCID: 8343736
42. Rodgers R, Latif Z, and Copland M (2012) How I manage priapism in chronic myeloid leukaemia patients Br J Haematol 158(2) 155–164 https://doi.org/10.1111/j.1365-2141.2012.09151.x PMID: 22571386
43. Ponniah A, Brown CT, and Taylor P (2004) Priapism secondary to leukemia: Effective management with prompt leukapheresis Int J Urol 11(9) 809–810 https://doi.org/10.1111/j.1442-2042.2004.00872.x PMID: 15379953
44. Bintoro SUY, Romadhon PZ, and Suryantoro SD, et al (2021) Case report: priapism as the clinical presentation of chronic myeloid leukemia in accordance with reports created during last twenty years: a case report and literature review F1000Res 10 571 https://doi.org/10.12688/f1000research.53365.1
45. Veljković D, Kuzmanović M, and Mićić D, et al (2012) Leukapheresis in management hyperleucocytosis induced complications in two pediatric patients with chronic myelogenous leukemia Transfus Apheresis Sci 46(3) 263–267 https://doi.org/10.1016/j.transci.2012.03.012
46. Morton NS (2007) Management of postoperative pain in children Arch Dis Child Educ Pract 92(1) 14–19
47. Simonini A, Brogi E, and Cascella M, et al (2022) Advantages of ketamine in pediatric anesthesia Open Med 17(1) 1134–1147 https://doi.org/10.1515/med-2022-0509
48. Zipper R, Younger A, and Tipton T, et al (2018) Ischemic priapism in pediatric patients: spontaneous detumescence with ketamine sedation J Pediatr Urol 14(5) 465–466 https://doi.org/10.1016/j.jpurol.2018.05.005 PMID: 29936034
49. Villalonga A, Beltran J, and Gomar C, et al (1985) Ketamine for treatment of priapism Anesth Analg 64(10) 1033–1034 PMID: 4037388
50. Gale AS (1972) Ketamine prevention of penile turgescence JAMA 219(12) 1629 https://doi.org/10.1001/jama.1972.03190380055019 PMID: 5066823
51. Seideman CA and Gitlin JS (2017) T-shaped shunt with intracavernosal tunneling for a pediatric case of refractory ischemic priapism Urology 110 220–222 https://doi.org/10.1016/j.urology.2016.08.047
52. Clark AJ, Hsu P, and Darves-Bornoz A, et al (2018) Priapism in a 13-year-old boy. Pediatrics in review Am Acad Pediatr 39 617–619
53. Vishnu S, Sidharth M, and Vansh B, et al (2023). Priapism as presenting manifestation in a case of pediatric CML, international clinical case reports and reviews BioRes Sci 1(1) 1–3
54. INFONA (2011) 52nd National Conference of Indian Society of Hematology & Transfusion Medicine (ISHTM) 2011 Indian J Hematol Blood Transfus 27(4) 185–289
55. Khan A, Shafiq I, and Shah MH, et al (2018) Chronic myeloid leukaemia presenting as priapism: a case report from Khyber Pakhtunkhwa J Pak Med Assoc 68(6) 942–944 PMID: 30323364
56. Hazra SP, Priyadarshi V, and Gogoi D, et al (2013) Pediatric priapism: a rare first manifestation of leukemia APSP J Case Rep 4(3) 39
57. Gupta A, Seth T, and Gupta A (2009) Successful use of terbutaline in persistent priapism in a 12-year-old boy with chronic myeloid leukemia Pediatr Hematol Oncol 26(1) 70–73 https://doi.org/10.1080/08880010802435146 PMID: 19206011
58. Abbott LS, Moineau G, and Johnston DL (2008) Case 1: an unusual cause of headaches and priapism in a teenager Paediatr Child Health 13(4) 299–301 https://doi.org/10.1093/pch/13.4.299
59. Bhatia P, Arya LS, and Chinnappan D, et al (1992) Priapism in chronic myelogenous leukemia Indian J Pediatr 59(1) 130–132 https://doi.org/10.1007/BF02760917 PMID: 1612657
60. Purohit AHL, Sarangi S, and Kumar D, et al (2021) Is priapism a common presentation of chronic myeloid leukemia in an adolescent patient? Cardiovasc Hematol Disord-Drug Targets 21(2) 147–148 https://doi.org/10.2174/1871529X21666210331142330 PMID: 33797372
61. Narendra R, Shankar JL, and Jain S, et al (2011) Priapism in teenager chronic myelogenous leukemia: a rare occurrence Asian J Pharm Hea Sci 1(4) 225–226
62. Sareen R, Kapil M, and Malpani BK (2018) Priapism: a rare presentation of CML J Hematol Oncol Forecast 1 1–3
63. Güzelküçük Z, Kaçar D, and Demir R, et al (2019) Priapism: a rare presentation of precursor B-cell acute lymphoblastic leukemia Turk J Pediatr 61 311–312 https://doi.org/10.24953/turkjped.2019.02.029
64. Werther R, Oakley E, and Heath JA (2004) Priapism as a presentation of T-cell acute lymphoblastic leukaemia in a child Emerg Med Australas 16(5–6) 425–427 PMID: 15537405
65. Mentzel HJ, Kentouche K, and Doerfel C, et al (2004) High-flow priapism in acute lymphatic leukaemia Pediatr Radiol 34(7) 560–563 https://doi.org/10.1007/s00247-003-1124-1 PMID: 15205837
66. Albagshi M (2020) Successful conservative management of priapism in a child with acute lymphoblastic leukemia Egypt J Haematol 45(4) 217 https://doi.org/10.4103/ejh.ejh_34_20 45(4) 217
