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Case Report

Case Report: Priapism as The Clinical Presenting Feature of Chronic Myeloid Leukemia: Case Report and 20-Year Literature Review

[version 2; peer review: 1 approved, 1 approved with reservations]

Previously titled: ‘Case Report: Priapism as The Clinical Presenting Feature of Chronic Myeloid Leukemia: Case Report and 20-year Literature Review’

PUBLISHED 13 Jan 2022

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Priapism is a urological emergency due to persistence of an erection lasting more than 4 hours, whether or not it is related to sexual influence.1 Priapism is a rare condition with an incidence of 1–5 cases per 100,000 people per year. Penile erection in priapism is regularly painless. There are two types of priapism, which are low-flow priapism and high-flow priapism. Low-flow priapism is provoked by a pathological condition of low venous blood flow causing stasis in the penile vessels. This condition is an emergency condition that can result in cell damage and fibrosis, thus it often requires immediate therapy. Meanwhile, high-flow priapism is caused by increased blood flow to the sinusoid arteries without offsetting the flow to the veins. One of the causes of high-flow abnormalities is penile injury, while low-flow priapism is commonly caused by blood disorders such as sickle cell anemia and chronic myeloid leukemia (CML).24

Priapism accounts for 20% of the hematological abnormalities while 1–5% of priapism are due to leukemia. The theory behind a priapism is the dysregulation of nitric oxide (NO) in penile vascularization. This occurs due to changes in NO synthase enzyme activity which decrease NO production by the corpora cavernosa. This ischemic condition induces platelet aggregation, thrombus, and tissue damage. Decreased NO interferes with smooth muscle tone and generates the priapism. Hyperviscosity conditions due to leukocytosis and adenosine-opiorphins abnormalities is also involved in this condition.1

Currently, the approach to treat CML patients with priapism uses a combination of systemic therapy (chemotherapy with hydroxyurea or tyrosine kinase inhibitors and leukapheresis) and local intracavernosal therapy. Some cases with late manifestations cause erectile dysfunction, gangrene and penile abscess.5 This case report and review aims to discuss the clinical characteristics and outcomes of CML patients who experience priapism.


An 18-year-old unmarried male student, presented at the ER complaining of persistent erection of the penis. The patient complained of persistent erected penis for 20 days before admission. There was no phase without an erection in between. Previously, there was neither history of trauma to sexual stimulation, nor consumption of certain drugs. The patient also complained of mild genital pain along with the onset of erection. There were no complaints about discoloration of the penis; becoming reddish, bluish, or pale, also there was no numbness. The patient could urinate normally (see Figure 1).


Figure 1. Penis at day 2, day 6 (after intracavernosal blood aspiration), and day 9.

The patient complained of tinnitus in his right and left ears for 15 days accompanied by blurred vision. The patient also felt that his left side of stomach was slowly enlarging for 5 months. There was no bleeding and fever. Before coming to the ER, the patient was hospitalized at the regional hospital and received a blood transfusion and was diagnosed with a blood disorder.

Physical examination revealed no anemia and icterus. The spleen was palpable showing Schuffner 4 and Hackett 3. There was no enlargement of the lymph nodes. His laboratory findings were hemoglobin 10.4 g/dL; leucocytes 421,000 cells/mm3; platelets 407,000 cells/mm3; white blood cells differential 4.3/6.8/81.3/4.9/2.7; blood urea nitrogen 9 mg/dL; serum potassium 0.5 mg/dL, uric acid 6.5 mg/dL. Peripheral blood smear showed normochromic anemia, normocytic anisopoikilocytosis, leukocytosis (3% myeloblasts, 6% promyelocytes, 4% myelocytes, 2% metamyelocytes, 5% stab neutrophils, 63% segment neutrophils, 4% eosinophils, 6% basophils, 5% lymphocytes, 2% monocytes, atypical lymphocytes (+)) concluded as CML. The patient received hydroxyurea 2000 mg once daily at night, paracetamol 500 mg TID, and an urgent leukapheresis.

The patient underwent leukapheresis once per day (three times since initial admission) with gradual improvement. Unfortunately, on the fourth day of treatment the patient felt a penis erection again with pain on a scale of 0–5. Local examination of the genitalia showed a maximal erected penis, with no discoloration indicative of hyperemia, cyanosis, or pallor. Blood gas analysis showed pH 6.95, pCO2 64 mmHg, HCO3 14 mEQ/L, BE -18 unit. We concluded that the patient had ischemic priapism. Therefore, the patient underwent intracavernous aspiration producing 150 mL blood. Not long after that, the patient’s penis returned to an erection with bleeding from the puncture wound. We then decided to give leukapheresis to the patient.

On the eighth day of treatment, the erection improved with pain scale of 1. Quantitative BCR–ABL examination showed a positive result of 65%, thus the administration of hydroxyurea was stopped and replaced by imatinib 400 mg once daily at night. On the twelfth day of treatment, the erection completely resolved and the patient was successfully discharged from the hospital.


This review presents data on patients who have priapism due to CML (see Table 1). Priapism occurred in the age ranging from 9–53. Patients usually had episodes of priapism for 18 h to 7 days. Not all patients with priapism showed a typical clinical examination of CML in the form of splenomegaly, but all of these patients had a hyperleukocytosis profile with a leukocyte count >200,000 cells/mm3. Some of them are equipped with data of peripheral blood smear with excessive blast and identification of BCR–ABL gene. A study by Minckler et al. was the only one reporting a resolved erection with a cold shower, whilst most other cases needed medical intervention.6 Although the duration of symptoms varied, four cases reported complications following an episode of priapism. Patients with unfavorable outcomes once received hydroxyurea, imatinib but failed to undergo urological emergency therapy such as intra-cavernosa aspiration, surgical intervention, and embolization.

Table 1. Case report review from last than twenty years.

No Author Country Year Age Duration of priapism Diagnosis of CML Treatment of CML Treatment of priapism Outcome of the treatment
1 Gaye et al.4 Senegal 2020 46 48 hours White Blood Cell: 526000/mm3, Platelets: 412000/mm3, Myelogram result: bone marrow hyperplasia. Karyotyping: Translocation between chromosomes 9 and 22 Imatinib (the dosage wasn’t mentioned) Aspiration of corpora cavernosa, injection of phenylephrine, hydrocycarbamide Success
2020 9 36 hours White Blood Cell: 82000/mm3, Platelets: 81000/mm3, BMA: acute myeloid leukemia Vincristine and Prednisolone penile skin refrigeration, rehydration, puncture of corpora cavernosa, injection of phenylephrine Success
2 Rajabto et al.9 Indonesia 2020 44 4 days physical exam: pale skin, conjunctival pallor, leukemic retinopathy in both eyes. Schuffer 2. IV fluid, Allopurinol 300 mg, Sodium bicarbonate 500 mg 3 times daily, hydrocyurea 1 gram three, Imatinib 400 mg times a day aspiration of penile corpus, injection of epinephrine suffered ED
Labs: anemia, hyperleukocytosis, microcytic hypochromic, anisopoikilocytosis, fragmentocytes, polychromic erythrocytes, a left shift, platelet count (355,000/μL), and hyperleukocytosis (399.560/μL).
Positive BCR-ABL1
BMA: hypercellularity
3 Dhar et al.11 India 2019 52 4 hours Physical examination: massive splenomegaly of 8 cm below the left costal margin along with hepatomegaly of 3 cm below right costal margin. Hydroxyurea 500 mg TDS, Imatinib OD, Allupurinol 300 mg OD, adequate hydration needle aspiration –> didn’t work, went for Winters procedure Success
Blood count: left sided granulopoeis, total leucocyte count of 239×109/L and platelet count of 625×109/L.
BMA: findings of CML
positive translocation of BCR-ABL
4 Becerra et al.12 Mexico 2018 52 6 day evolution WBC: 282.000, platelets: 368×103/mm3 dastinib 100 mg/day+G15 corpora cavernosa irrigation and surgery penis shunts Success
BMA: acute phased CML
translocation t (9:22)(q34;q11.2) with P210 BCR-ABL1 fusion transcriber
5 Khan et al.13 Pakistan 2018 16 264 hours Leukocyte count: 614.8×109, platelets 709×1012/L, peripheral smear: myeloid hyperplasia, neutrophilia. BMA: myeloid hyperplasia. Detection of BCR-ABL Hydroxyurea, allopurinol Glans-cavernosal shunt Achieved detumescence, No info on ED
6 Qu et al.14 China 2018 18 72 hours Hepatosplenomegaly 2-3 cm under arcus costae, blood count: white blood cell (WBC) 257×109/L and platelets (PLT) 5450×109/L Imatinib Caverosa-corpus spongiosum shunt No ED at 3 months follow up
7 Clark et al.15 USA 2018 13 3 days Blood count: WBC count of 350,000/mL (350×109/L) and platelet count of 450×103/mL (450×109/L). Flow cytometry of blood: granulocytosis with no increase in blasts leukapharesis, IV fluids, hydroxyurea, allupurinol, Imatinib phenylephrine injection, three times corporeal irrigation improved with phallus rigidity and tenderness
BMA: Philadephia chromosome
8 Kumar et al.16 India 2018 47 5 days Hepatosplenomegaly, WBC: 279×109, 91.2%BCR Hydroxyurea, Imatinib Aspiration and irrigation with phenlyephrine, Winter’s T Shunt Successful treatment
42 7 days Splenomegaly 6 cm below costal margin, WBC: 390×109/L, 70,7% BCR-ABL ratio Hydroxyurea, Imatinib Aspiration and irrigation Successful treatment
28 6 days No hepatosplenomegaly, WBC: 206×109/L, 75.3% BCR-ABL ratio Hydroxyurea, Imatinib Aspiration and irrigation with phenlyephrine, Winter’s T Shunt Successful treatment
9 Sun et al.5 USA 2018 27 8 years, persistent erection 9 hours Labs: anemia, WBC 450,010, Platelets 509,000/mm3 BMA: 2% blasts, hypercellular bone marrow, granulocytic hyperplasia, small megakaryocytes. BCR-ABL did not reveal clonal evolution. Leukapheresis, hydoxyurea 500 mg daily, allopurinol 300 mg daily, Imatinib 400 mg daily, Corporal bpody aspiration, 1 dose of phenylephrine injection Successful treatment
10 Huei et al.17 Malaysia 2018 28 48 hours hepatomegaly 2cm below right costal margin, splenomegaly, anemia, WBC 294×109, platelets: 94×109/L Peripheral blooad smear: hyperleucocytosis, blast cells Hydroxyurea, allupurinol, intravenous Cytarabine Intracavernosal aspiration, phenylephrine irrigation–> detumescent –> reccurent erection –> corpoglandular shunt Successful treatment
11 Minckler et al.6 USA 2017 18 3 month intermittent WBC: 588×103/uL, platelets: 109×103/uL Hydroxyurea transtition to imatinib 400 mg daily Penile irigation and aspiration Success
peripheral blood: hyperleukocytosis with absolute neutrophilia and
a peripheral blast count of 2%.
bone marrow
aspirate and biopsy: hypercellular marrow with 4% blasts
FISH analysis: translocation t(9:22)
12 Nerli RB et al.7 India 2016 19 duration: 24 hours WBC 296800, platelet 936,000/mm3, BMA: hypercellular, increased megakaryocytes Hydroxyurea 1.5 gram daily, Imatinib 40 mg daily Allupurinol 300mg daily per oral Irrigation, decompression Successful
13 Ergenc H et al.18 Turkey 2015 18 duration: 72 hours Hepatosplenomegaly 2-3 cm under arcus costae, anemia, WBC 100.000, platelets 1,002,000/mm, peripheral blood smear: immature leukocytes. BMA: hypercellularity with myeloid hyperplasia, positive BCR-ABL translocation Imanitib 400 mg once daily, allopurinol 300 mg once daily, leukapharesis not mentioned Success
14 Shaeer et al.2 Egypt 2015 21 6 days palpable splenomegaly, WBC 410000, Philadelphia chromosome translocation Leukapharesis, Imatinib 400 mg daily failed several cavernosal aspiration and injection of epinephrine –> penile prosthesis No complication throughout 6 months-follow up
15 Osorio et al.19 Spain 2014 24 14 hours, the second episode. The first episode was 4 months ago WBC: 177.15×109, platelet was not mentioned, cytogenic diagnosis: showing CML Imatinib Corpora cavernosa aspiration, intracavernosa fenilefrin injection not mentioned
29 6 hours, the second episode. The first episode was less than a month ago WBC: 402.24×109, platelet was not mentioned positive BCR-ABL hyrdoxyurea Corpora cavernosa aspiration, intracavernosa fenilefrin injection not mentioned
16 Hazra et al.20 India 2013 14 24 hours Splenomegaly 6 cm below the left costal margin, anemia, WBC 226900, platelets 310,000/uL, Peripheral blood smear: immature leukocytes in various stages. BMA: CML. Hydroxyurea 50 mg/kgBB/day, Allupurinol 300 mg/day Cavernosal aspiration and phenylephrine irrigation No recurrence at 2-months-follow-up
17 Veljkovic et al.21 Serbia 2012 16 24 hours Splenomegaly 4 cm below costal margin, WBC 320×109/L, Platelet (Plt) 417×109/L BMA: extreme hypercellularity, BCR/ABL positive leukapharesis, cytoreductive chemotherapy leukapharesis no follow up
18 Paladino et al.3 Spain 2011 16 48 hours Splenomegaly, WBC 312.000, PLT: 60.000/mm3 BMA: showing CML no mention Corpora cavernosa drainage Erectile dysfunction
19 Gupta et al.22 India 2009 12 48 hours Hepatosplenomegaly below the costal margins, anemia, WBC: 346×109/L, platelet count of 40,000/mm3, peripheral blood smear: immature myeloid leukocytosis. Cytogenesis: philadelphia chromosome. BCR-ABL transcript was positive hydroxyurea 4g/day IV fluid 3L/day, allopurinol, Imatinib 400mg/day, leukapharesis Terbutaline 0.125 mg subcutaneously Resolved by 24 h
20 Ilais Tazi23 Morocco 2009 33 duration: 22 hours Palpable splenomegaly 4 cm below left costal margin, WBC: 400000/mm3, platelets 1200000/mm3. Peripheral blood smear: immature leukocytes. Karyotyple analysis: Ph1 chromosome, myeloid hyperplasian in the bone marrow. Imatinib Aspiration Success
21 Castagnetti et al.24 Netherland 2008 9 several days splenomegaly, anemia, WBC: 509×109/L, philadelphia chromosome, BCR-ABL + Hydroxyurea 1.5mg/m2/day, Cyclophosphamide 250 mg/m2/day for 2 days, leukopharesis cytoreduction, antibiotics, anticoagulants Fully resolved after 1 month
9 96 hours mild splenomegaly Hydroxyurea 1g/m2/day LMWH 90 units/kg SQ BID for 1 month, metamizole fully resolved after 3 months
9 9 hours hepatosplenomegaly Cyclophosphamide 250 mg/m2/day for 2 days, leukapharesis LMWH 90 units/kgBB SQ BID for 9 days, metamizole, morphine fully resolved after 20 days
22 Yoshida et al.25 Japan 2007 29 48 hours WBC 263000 Imatinib mesylate Winter procedure no evidence of recurrent
23 Lopez et al.26 Spain 2004 29 10 hours WBC 414×109/L, BMA: hypercellularity, PLT: 1100 × 109/L corpora cavernosa aspiration, phenylephrine injection corpus cavernosum aspiration, fenilefrin injection Successful treatment
24 Ponniah et al.27 United Kingdom 2004 19 18 hours WBC 513×109/L Leukapharesis failed cavernosal aspiration + leukapharesis No ED on follow up
25 Dogra et al.28 India 2003 18 10 days hepatosplenomegaly, anaemic, WBC 320000, PLT was not mentioned Intravenous hydration, furosemide, sodium bicarbonate, hydroxyurea, allopurinol, leukapharesis Winters Procedure impotent and enlarged penis at 3-months follow up
26 Meng-Wei Chang et al.8 Taipei 2003 21 19 hours Hepatomegaly 6 cm below right arcus costae, Splenomegaly 7 cm below left arcus costae, anemia, WBC 216800, Platelet 1746,000/mm3 Interferon alfa-2a (6MIU/vial), allopurinol 300 mg daily Aspiration, epinephrine irigation Success
27 Guerra et al.29 Spain 2002 53 12 hours WbC 968×109/L Hydroxyurea Corpora cavernosa aspiration Successful treatment
28 Murayama et al.30 Japan 2001 14 4 days WBC 510000, BMA: myeloid hyperplasia, karyotype analysis: chromosome Ph1 urokinase, hydroxyurea embolization of bilateral pudendal artery Reduced sexual potency
29 Rojas et al.31 Chilli 1998 22 duration: 36 hours none Leukapharesis Surgical intervention Unsuccessful (post treatment sexual dysfunction)

The patient in our study was 18 years old. However, based on the literature, patients in every age group are at risk of developing priapism. There are two peaks in the age distribution that tend to experience this condition. The peak in earlier age is between 5 and 10 years, especially in patients with sickle cell disease. Meanwhile, the second peak is at sexually active phase between 20 and 50 years. Apart from hypercoagulability, this condition may also be related to the abuse of erectile drugs.7

History and physical examination are important when encountering cases of priapism. Laboratory tests are required to check for impaired coagulation and serum electrolytes. Some patients who are at high risk for priapism include users of intracorporal injection therapy for erectile dysfunction, coagulation disorders such as sickle cell disease and CML.2,4 In CML, hyperleukocytosis is thought to be the prime cause of priapism. The main mechanism is the aggregation of leukemic cells in the corpora cavernosa and dorsal veins of the penis. Other than that, mechanical pressure in the abdominal veins due to the enlargement of the spleen might also increase the risk.1

The data needed in the management of patients with this case are erection duration, pain scale, trauma, complete blood count, peripheral blood smear, penile blood gas analysis, bone marrow and polymerase chain reaction for BCR–ABL1 if necessary.1,2,4 In CML, the most common type of priapism is the ischemic one (veno-occlusive). Patients usually complain of painful, rigid erection, with reduced to no cavernous blood flow at all. Priapism that lasts for more than 4 hours indicates a compartment syndrome and may require emergent medical intervention.8

The American Urological Association recommends that systemic treatment of an underlying disorder should not be the only one therapy for ischemic priapism. In this case, the patient had an erectile episode since 20 days before the admission. This phenomenon was likely due to the compartment syndrome, hence the intra-cavernous aspiration was required.1

The intra-cavernous aspiration procedure can be accomplished by giving the anesthetic injection first under the symphysis pubis. The penis is tied with a tourniquet followed by insertion of a 16–18-Gauge bivalve intravenous catheter into the corpus cavernosum. When the two corpora are fused, aspiration of 20–30 mL of blood can be undertaken. This procedure has 30% chances of success.8,9

Systemic therapy is often used to reduce hyperviscosity is cytoreductive therapy such as high-dose hydroxycarbamide and tyrosine kinase inhibitors (TKI) with or without apheresis procedures. Hydroxycarbamide can be given 2–6 grams divided into four doses per day. This can reduce leukocytes by almost 60% in 24–48 h. In addition, TKI, such as imatinib, can be administered as soon as the diagnosis is confirmed. The recommended dose of imatinib is 400 mg once daily in the chronic phase, 600–800 mg once daily in the accelerated phase, and 800 mg once daily in a blast crisis.9 Generally, IRIS study describes the effectiveness of imatinib therapy for complete hematological response (CHR), major cytogenetic response (McyR) and complete cytogenetic response (CcyR).4

Leukapheresis can promote a rapid decrease in intravascular leukemic cells, improve tissue perfusion and prevent leukostasis (generally show pulmonary and central nervous system manifestations). Once leukapheresis is given, it possibly can reduce the leukocyte count by 30–60%. However, compared to the chemotherapy, several previous studies have shown that this procedure had high all-cause mortality. According to 2016 apheresis guidelines, category 2 (second-line therapy) is recommended for grade 1B of acute myeloid leukemia (strong recommendation, moderate quality evidence), while category 3 (unclear role of apahresis) is recommended for acute lymphoblastic leukemia cases grade 2C (weak recommendation, low quality evidence). In this guideline, leukapheresis is not recommended for chronic myeloid leukemia.10 Several cases of priapism in this case review reported a successful combination of leukapheresis with systemic oral CML therapy. A study by Rojas et al. was the only one reporting a failed leukapheresis.

This case report and review presents a comparative presentation of patient characteristics, clinical characteristics of CML, laboratory profile, and therapeutic intervention for CML with priapism. Clinical presentation and early intervention are pivotal keys to achieve favorable outcome and prevent complications. Systemic intervention combined with intraurethral therapy may add the success rate (see Figure 2).


Figure 2. Treatment and outcome from priapism and CML.

Eventually, further discussion and study on other causes of priapism is essential as a meta-analysis stated that priapism might also be related to lymphoproliferative disorders.32


Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient.

Data availability

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

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