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Year : 2021  |  Volume : 26  |  Issue : 3  |  Page : 205-207

Rare presentation and initial management of ischemic priapism in two cases of chronic myeloid leukemia

1 Division of Urology, Department of Surgery, Ahmadu Bello University/Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria
2 Division of Clinical Haematology, Department of Medicine, Ahmadu Bello University/Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria
3 Department of Obstetrics and Gynaecology, Ahmadu Bello University/Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria

Date of Submission06-Jun-2020
Date of Decision14-Oct-2020
Date of Acceptance11-Jan-2021
Date of Web Publication20-Apr-2021

Correspondence Address:
Musliu Adetola Tolani
Division of Urology, Department of Surgery, Ahmadu Bello University/Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijmh.IJMH_40_20

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Priapism is a rare clinical presentation in patients with chronic myeloid leukemia. Treatment involves multidisciplinary team efforts of the urologist and haemato-oncologist. We report two cases of priapism: a 20-year-old male with chronic myeloid leukemia who presented with priapism as a component of hyperviscosity syndrome and a 26-year-old previously healthy, non-sexually active male who had priapism as his first presentation of chronic myeloid leukemia, and the role of specialists in the management of these patients.

Keywords: Chronic myeloid leukemia, management, presentation, priapism

How to cite this article:
Tolani MA, Sudi A, Jamoh BY, Afolayan AO, Opoola AA, Shehu FA. Rare presentation and initial management of ischemic priapism in two cases of chronic myeloid leukemia. Int J Med Health Dev 2021;26:205-7

How to cite this URL:
Tolani MA, Sudi A, Jamoh BY, Afolayan AO, Opoola AA, Shehu FA. Rare presentation and initial management of ischemic priapism in two cases of chronic myeloid leukemia. Int J Med Health Dev [serial online] 2021 [cited 2021 Dec 9];26:205-7. Available from: https://www.ijmhdev.com/text.asp?2021/26/3/205/313950

  Introduction Top

Priapism is defined as a complete or partial penile erection that lasts more than 4 hours beyond sexual stimulation and orgasm or is unrelated to sexual stimulation.[1] It is a urological emergency that is commonly secondary to sickle cell disease and aphrodisiac use.[2],[3],[4] However, in rare cases, adult patients with leukemia can present with this condition. There are sparse data on its manifestation as a component of hyperviscosity syndrome in this haematological malignancy, its rare occurrence as the initial presenting feature of chronic myeloid leukemia, or the approach to emergency treatment of priapism in these patients.[5],[6] The two cases in this report highlight these rare clinical presentations and the role of urologists and haemato-oncologists in the management.

  Case Reports Top

Case report 1

A 20-year-old male, with a diagnosis of chronic myeloid leukemia on bone marrow aspiration by the clinical haematology unit of our tertiary center 1 year earlier, with poor compliance to prescribed cytoreductive agent (hydroxyurea 1 g twice daily), who now presented to the urological division in our emergency unit with a complaint of the first episode of progressively increasing painful penile erection of 5 days duration that started while he was sleeping. He did not have sickle cell anemia, previous history of perineal or back trauma, sexual activity, or use of erectogenic medications. Physical examination revealed an acute on chronically ill-looking young man with pallor but no fever or petechial hemorrhage. He, however, had significant lymphadenopathy in the axillary region, elevated blood pressure (140/100 mmHg), and massive splenomegaly of 20 cm below the left costal margin. External genitalia examination revealed an erect, turgid, tender phallus with a soft glans.

The diagnosis of neglected ischemic priapism with hyperviscosity syndrome on background chronic myeloid leukemia was made which warranted multidisciplinary management by urologists and haemato-oncologists. Complete blood count at presentation revealed hyperleukocytosis of 298×109/L, thrombocytopenia of 76×109/L, and anemia with packed cell volume of 21.5%. He was resuscitated by blood transfusion and then had corporoglanular shunt (Al-Ghorab) due to persistent pain with findings of dark blood aspirate from the corpora cavernosum and 80% on-table detumescence. He was also hyperhydrated and re-commenced on the earlier cytoreductive medications. Penile detumescence was sustained post-operatively, and the patient was continued on cytoreductive medications. However, he developed a deep surgical site infection 3 weeks after the corporoglanular shunt. Wound swab culture yielded Klebsiella spp., which was treated by abscess drainage, regular wound dressing, and antibiotics administration. He had post-priapism erectile dysfunction at the last visit 14 months after the procedure and had been counseled for semi-rigid penile prosthesis insertion.

Case report 2

A 26-year-old male was referred to the emergency ward with a 36-h history of sustained painful penile erection which developed spontaneously while on a long-distance journey. He also had decreased audition in the right ear with associated tinnitus and dizziness. This is the first episode of this presentation in patient’s life. He had no prior history of sexual activity, aphrodisiac use, trauma to the back or perineum, or sickle cell anemia. No previous recurrent fever or bleeding tendency. On examination, the patient was a young adult with no sickle cell habitus, in painful distress, not pale, and had no fever or petechial hemorrhage. There was, however, significant axillary lymphadenopathy, hepatomegaly of 6 cm below the right costal margin, and splenomegaly of 12 cm below the left costal margin. Examination of the external genitalia revealed an erect tender turgid phallus with a soft glans.

The diagnosis of ischemic priapism secondary to suspected hematological malignancy was made. Urgent complete blood count revealed hyperleukocytosis of 223×109/L, anemia with hemoglobin of 9.7 g/dL, and thrombocytopenia. There was a 10% band form, series of myeloid maturations, and numerous blast cells on peripheral blood film, while there was hyperkalemia of 6.7 mmol/L on serum electrolytes. His hemoglobin phenotype was AA on hemoglobin electrophoresis. The patient was resuscitated and had emergency corporoglanular shunt (Al-Ghorab) with findings of 50 mL of dark corporal blood aspirate. He had complete detumescence following the procedure. Post-operatively, he was placed on antibiotics, analgesics, and intermittent penile massage. He also had hyperhydration, exchange blood transfusion, and Allopurinol 300 mg daily to abate features of tumor lysis syndrome. Bone marrow aspiration confirmed the diagnosis of chronic myeloid leukemia. He was commenced on hydroxyurea 1 g three times daily as cytoreductive therapy. White blood cell count reduced to 99×109/L 2 months after commencement of therapy. At the last review 4 months after the shunt procedure, there was no recurrence of priapism.

  Discussion Top

Priapism is a rare condition with an incidence of 1.5 cases per 100,000 person-years.[1] Its development in patients with leukemia is quite uncommon as it occurs in only 1–2% of them with less than 20 cases described in the literature.[6] The two cases in this study developed this urological emergency in their twenties. This is similar to patients reported in the works of Shaeer et al. and Boongaling et al. and falls within the second peak age of occurrence of priapism (20–50 years) in variance with those of sickle cell disease patients, which tends to present earlier.[4],[7],[8] The late presentation of these patients could be attributed to poverty, ignorance, and poor health-seeking behavior of the patients.

The penile erection was painful in these patients suggesting an ischemic pathology which was further confirmed with the dark corporal blood aspirate during the shunt procedure. In these two patients, chronic myeloid leukemia was identified as the underlying cause, first on suspicion from the presence of splenomegaly, and later, following its confirmation on bone marrow examination as elucidated in the work of Chisick et al.[9] These cases are unique because it occurred in the setting of other manifestations (visual disturbance) of hyperviscosity syndrome in a known chronic myeloid leukemia patient (case 1) and as the debut presentation of chronic myeloid leukemia even in the absence of other clinical symptoms (case 2). It has been stated that leukocyte count greater than 100×109/L significantly increases the blood viscosity.[8] Thus, hyperleukocytosis, which leads to the accumulation of leukemic cells in the corpora cavernosa and dorsal vein of the penis, and the massive splenomegaly, which causes pressure on intra-abdominal veins, present in these patients could cause venous obstruction, subsequent ischemia, and penile fibrosis without early intervention.[5]

The management of priapism in chronic myeloid leukemia patients has been based on experience from case reports.[2] While systemic therapy of the underlying pathology is important to reduce white blood cell count and prevent tumor lysis syndrome, immediate intracavernosal treatment is necessary, as done in the two cases reported in this study, to relieve pain and halt further corporal damage, especially in the second case.[10] The two cases presented strengthen the premise for multidisciplinary management of these patients by urologists and haemato-oncologists.

  Conclusion Top

Priapism is an uncommon presentation in chronic myeloid leukemia. A high index of suspicion of this haematological malignancy is important when priapism occurs in a previously healthy, non-sexually active patient, with no previous morbidity, especially in those with the presence of splenomegaly. Combined urological and oncological treatment would ensure the holistic management of these patients.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Nerli RB, Magdum PV, Hiremath SC, Patil AY, Pai SV, Handigund RS, et al. Priapism—A rare presentation in chronic myeloid leukemia: Case report. Urol Case Rep 2016;4:8-10.  Back to cited text no. 1
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.  Back to cited text no. 2
Ajape AA, Kuranga IS, Babata AL, Abiola OO, Obiano SK, Sanni MA, et al. Priapism as an initial presentation of chronic myeloid leukaemia: 2 case reports. Sahel Med J 2008;11:65-7.  Back to cited text no. 3
  [Full text]  
Dhar J, Dhar J, Chhabra G, Khandelwal L, Khandelwal L, Batra A, et al. Priapism as a debut presentation of chronic myeloid leukemia. J Coll Physicians Surg Pak 2019;29:78-80.  Back to cited text no. 4
Sareen R, Kapil M, Malpani BK Priapism: A rare presentation of CML. J Hematol Oncol Forecast 2018;1:1-3.  Back to cited text no. 5
Huei TJ, Lip HT, Shamsuddin O A rare presentation of chronic myeloid leukaemia with priapism treated with corporoglandular shunting. Med J Malaysia 2018;73:420-2.  Back to cited text no. 6
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.  Back to cited text no. 7
Boongaling MEDC, Mortel SRC, Deala RP Priapism as a rare presentation of chronic myelogenous leukemia. Phillipine J Intern Med 2015;53:1-5.  Back to cited text no. 8
Chisick L, Seftel M, Kumar R Algorithm for initial management of priapism in chronic myeloid leukaemia. Br J Haematol 2012;159:250-1.  Back to cited text no. 9
Rodgers R, Latif Z, Copland M How I manage priapism in chronic myeloid leukaemia patients. Br J Haematol 2012;158:155-64.  Back to cited text no. 10


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