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Acta Bio Medica : Atenei Parmensis logoLink to Acta Bio Medica : Atenei Parmensis
. 2021 Jul 1;92(3):e2021193. doi: 10.23750/abm.v92i3.10796

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

Elrazi Ali 1, Ashraf Soliman 2, Vincenzo De Sanctis 3, Doris Nussbaumer 4, Mohamed A Yassin 4,✉
PMCID: PMC8343736  PMID: 34212918

Abstract

Background:

Priapism is defined as a penile erection that persists four or more hours and is unrelated to sexual stimulation. Priapism resulting from hematologic malignancy is most likely caused by venous obstruction from microemboli/thrombi and hyperviscosity caused by the increased number of circulating leukocytes in mature and immature forms. In patients with leukemia, 50% of cases of priapism are due to Chronic Myeloid Leukemia (CML). We present a systematic review of priapism in CML.

Acquisition of evidence:

An extensive literature research was carried out in PubMed, Google Scholar, SCOPUS, and Science Citation Index databases. The search included cases up to 4th August 2020.

Synthesis of evidence:

A total of 68 articles were found and included in our review, including 3 reviews from three different centers. We found 68 articles (102 patients; figure 1) and several case reports on priapism in CML. Priapism was noticed in some patients at the first presentation of CML. However, it was infrequently reported during the start of treatment, following the stop of medication and post-splenectomy. The mean age at presentation was 27.4 years, and the mean time from onset of priapism to the time to get medical attention (presentation) was 78.2 hours. The mean white blood cell count associated with priapism was 321.29x109/L, and the mean platelet count was 569 x10 9/L. The chronic phase of CML was the most common phase where priapism occurred. Most patients were Asian (>50%). Nearly a quarter of patients (27.4%) developed permanent erectile dysfunction.

Conclusions:

Priapism is a urological emergency requiring urgent multidisciplinary management to prevent erectile dysfunction. Because of the relatively rare occurrence of priapism in CML patients, there is no standard treatment protocol. (www.actabiomedica.it)

Keywords: Chronic myeloid leukemia, Chronic granulocytic leukemia, Erectile dysfunction, Priapism, Male fertility

Introduction

Hematological disorders are the leading cause of priapism, accounting for 20% of the cases and include SCD, hyperviscosity syndromes as seen with the myeloproliferative diseases, hypercoagulable states such as deficiencies of proteins C and S, antiphospholipid syndromes, And amyloidosis (1). Priapism is defined as a penile erection that persists four or more hours and is unrelated to sexual stimulation (2). The condition is classified into three subtypes: ischemic (low-flow), non-ischemic (high-flow), and stuttering (intermittent) priapism (3). Stuttering priapism is characterized by a recurrent and intermittent erection, frequently occurring in a specific patient population with SCD and less commonly with thalassemia (4). The clinical presentation of CML consists of lymphadenopathy (80%), asthenia, and fatigue (60%), spleen or liver enlargement (50%), weight loss (15–20%), and bleeding (10%), hyperleukocytosis about 80%, central nervous system affection (15%) kidney (5%) and priapism (≤ 3%) (5). Priapism due to hematological disorder is most likely due to venous obstruction from microemboli/thrombi as well as hyperviscosity due to an increased number of circulating leukocytes n mature and immature forms. Other accessory mechanisms are venous congestion of the corpora cavernosa secondary to mechanical pressure from the abdominal veins draining the spleen or infiltration of the sacral nerves or the central nervous system by leukemia cells (6). It is also seen that increased production of cytokines and adhesion molecules by leukemia cells result in endothelial cell activation and lead to increased sequestration of cells in the microvasculature (7).

Prolonged corporeal ischemia lasting more than 24 to 48 hours may result in varying extents Of irreversible fibrosis with endothelial and trabecula destruction of the erectile tissue and Subsequently in permanent erectile dysfunction and, therefore, is considered as a urologic Emergency. Reduced sperm count related to TKIS in patients with CML as well as priapism adversely affects the quality of life, particularly in populations where adolescent and young adults represent the majority of patients (8,9).

The objectives of this review were to: (a) assess the characteristics and risk factors of CML patients with priapism, (b) realize the common type of priapism in CML, (c) describe the management options adopted for priapism in CML, and (d) investigate the outcome and erectile dysfunction.

Acquisition of evidence

We searched the English literature (Google Scholar, PubMed, SCOPUS, and Science Citation Index databases) including original articles, reviews, case series, and case reports using the terms: “chronic myeloid leukemia,” “chronic myelogenous leukemia”, “chronic myelocytic leukemia” and “priapism”. A total of 68 articles were found and included in our review, among them 3 reviews were found from three different centers. The search included cases up to 4th August 2020.

Synthesis of evidence

We found 68 articles on priapism in CML (102 patients; figure 1) and several case reports (10-77). The youngest patient was 7 weeks old, and the oldest was 60 years old. The first case was reported in 1960 and the last in 2020. Most patients had priapism at their first presentation of CML. Three patients developed priapism after starting CML treatment, two after stopping treatment, and two were previously diagnosed. 80 /102 patients had splenomegaly (on clinical examination or by US abdomen), and 31/102 had hepatomegaly. The lowest white blood cell count (WBC) associated with priapism was 37 x10 9/L, and the highest was 782 x10 9/L.

Figure 1.

Figure 1.

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The PRISMA flow diagram detailing the cases of chronic myeloid leukemia (CML) presenting priapism.

Table 1 presents the characteristics of stuttering and ischemic priapism in patients with CML.

Table 1.

shows the characteristics of CML patients with priapism

Referenced Age in Years Priapism from onset to presentation to the hospital First presentation or previously Type of priapism WBC x10^9/L PLT x10^9/L HB g/dL Splenomegaly/ hepatomegally, below costal margin in cm or if presdnt stage of CML
10 18 72 hours Yes ischemic low flow 100 1,002 6 spleen 2-3 cm chronic phase
11 15 2 days Yes ischemic low flow 135 197 9 spleen to the umbilicus chronic phase
12 52 4 hours Yes ischemic low flow 239 625 8.9 spleen 8cm liver 3 cm chronic phase
13 19 18 hours Yes ischemic low flow 513 NA NA N/A chronic phase
14 38 30 hours Yes ischemic low flow 378 155 10.5 spleen 18 cm chronic phase
15 52 6 days Yes ischemic low flow 282 368 10 N/A chronic phase
16 21 72 hours Yes ischemic low flow 619 N/A 7.4 spienomegally chronic phase
17 30 9 days Yes ischemic low flow 261 86 9.2 spleen 10 cm chronic phase
18 29 3 days Yes ischemic low flow 366.34 622 6.7 spleen 5 cm, liver 2 cm chronic phase
19 20 4 hours Yes ischemic low flow 158 N/A 10.9 spleen 8 cm chronic phase
20 18 14 hours Yes ischemic low flow 363 527 9.7 spleen 9 cm accerated
21 55 2 days Yes ischemic low flow 420 280 9.5 spleen up to umbilicus , liver 2 cm chronic phase
22 17 NA Yes ischemic low flow 377,31 730 11.3 Hepatosplenomegaly Blasts+ Promyelocytes-12% chronic phase
23 21 19 hours Yes ischemic low flow 216 1746 8.3 spleen 7 cm, liver 6 cm chronic phase
24 28 2 days Yes ischemic low flow 294 94 6.6 spleen 2 chronic phase
25 22 4 days Yes ischemic low flow 218.6 324,2 8.2 spleen 6 chronic phase
26 21 6 days Yes ischemic low flow 410 N/A N/A spleen 3 fingers chronic phase
27 18 75 hours Yes ischemic low flow 323 N/A N/A N/A chronic phase
28 18 7 days Yes ischemic low flow 257 5450 N/A spleen 2-3 chronic phase
29 18 4 days Yes ischemic low flow 144 350 9.6 spleen 9-10 cm liver 2-3 cm chronic phase
30 30 16 hous after stopping ischemic low flow 210 45 10.3 huge splenomegaly chronic phase
31 18 5 days Yes ischemic low flow 215 470 6.9 spleen 10 cm chronic phase
32 18 10 days after start of treatment ischemic low flow 320 normal 6.5 hepato-splenomegaly. blast crisi
33 21 8 hours Yes ischemic low flow 316 670 8.3 liver palpable 6 cm , spleen 7 cm chronic phase
33 55 12 hours Yes ischemic low flow 282 260 9 spleen 7 cm chronic phase
34 36 5 days diagnosed at 33 ischemic low flow N/A N/A N/A N/A chronic phase
25 33 22 hours Yes ischemic low flow 400 1 200 10.53 spleen 4 cm chronic phase
36 16 11 days Yes ischemic low flow 614.8 907 5.7 liver 2cm spleen 4cm blast crisis
37 30 20 hours Yes ischemic low flow 285 462 11.2 spleen 2cm liver 3 cms chronic phase
37 25 2 days Yes ischemic low flow 670 320 11.3 spleen 1 cms chronic phase
37 28 7 hours Yes ischemic low flow 441 422 7 spleen 15 cms chronic phase
38 14 24 hours Yes ischemic low flow 226.9 310 9.9 spleen 6 cm chronic phase
39 16 NA Yes ischemic low flow 320 417 11 spleen 4 cm chronic phase
40 25 16 hour Yes ischemic low flow 501 570 269 10.3 no organomegally chronic phase
41 9 9 hours Yes ischemic low flow 274 1235 8.2 spienomegally blast crisis
42 55 8 hours Yes ischemic low flow 184 277 9 spleen 3 cm liver 2 cm chronic phase
43 23 3 days Yes ischemic low flow 660 321 8 hepatosplenomegaly chronic phase
44 47 5 days Yes ischemic low flow 297 N/A N/A hepatosplenomegaly chronic phase
44 42 7 days Yes ischemic low flow 390 N/A N/A spleen 6 cm chronic phase
44 28 6 days Yes ischemic low flow 206 N/A N/A no hepatosplenomegally chronic phase
45 7 weeks 5 days Yes ischemic low flow 37 344 10 liver and spleen were palpated below the costal margin. blast crisis
46 22 8 hours Known CML, stopped medications ischemic low flow 157 670 5.4 hepato-splenomegaly chronic phase
47 43 48 hours NA? ischemic low flow N/A N/A N/A N/A blast crisis
48 37 96 hours Yes ischemic low flow 150 215 13.9 N/A chronic phase
49 22 N/A after day 4 of treatment ischemic low flow 297 N/A N/A hepatosplenomegaly chronic phase
50 29 deveoped priapism after starting treatment busalfan and allopyranol in the 5th hospital admission 5th day ischemic low flow 296 800 N/A spleen 4 cm chronic phase
51 46 duration after presentation 22 days Yes ischemic low flow 782 400 N/A spleen 14 cm chronic phase
51 42 nonsustained Yes ischemic low flow 367 179 N/A spleen 9 cm chronic phase
51 42 24 hr Yes N/A 402 500 N/A spleen 10 cm chronic phase
51 18 4 dasys Yes N/A 451 726 N/A spleen 18cm chronic phase
52 45 year 18 hrs Yes N/A N/A N/A N/A N/A chronic phase
52 58 48 hrs Yes N/A N/A N/A N/A N/A chronic phase
52 40 12 hrs Yes N/A N/A N/A N/A N/A chronic phase
52 38 29 hr Yes N/A N/A N/A N/A N/A chronic phase
52 60 6 hrs Previously diagnosed N/A N/A N/A N/A N/A chronic phase
52 39 72 hrs Yes N/A N/A N/A N/A N/A chronic phase
53 9 3 Days Yes N/A 155 N/A N/A N/A chronic phase
53 11 5 days Yes N/A 210 N/A N/A N/A chronic phase
53 13 4 days Yes N/A 120 N/A N/A N/A chronic phase
53 10 3 days Yes N/A 200 N/A N/A N/A chronic phase
53 8 2 days Yes N/A 170 N/A N/A N/A chronic phase
54 22 6 days Yes N/A 580 N/A 7.2 spleen 22 cm, liver 10 cm chronic phase
54 30 8 days Yes N/A 300 N/A 6 hepatosplenomegally chronic phase
55 28 11 days Yes N/A 400 adequate 11.75 spleen 13 cm chronic phase
56 26 N/A Yes ischemic low flow 365 625 N/A spleen 17 cm liver 4 cm chronic phase
57 36 N/A Yes ischemic low flow 231 113 12 hepatosplenomegaly by US chronic phase
58 8 1 day Yes ischemic low flow 430 200 9 liver 1 cm, spleen down to the left inguinal ligament chronic phase
59 12 3 days Yes ischemic low flow 172 N/A N/A spienomegally chronic phase
60 45 2 days Yes ischemic low flow 410 350 6 massive spleenomegally chronic phase
61 22 5 days Yes iscemic low flow 200 increased 7.5 spleen 15 cm liver just palpable chronic phase
62 12 2 days Yes stuttering - ischemic 460 350 8.2 spleen 22 cm and hepatomegaly 4 cm. chronic phase
63 24 5 days Yes stuttering - ischemic 207 545 10 spleen 8 cm chronic phase
64 30 8 days Yes stuttering - ischemic 240 186 7.5 liver 1 cm spleen 6 cm chronic phase
64 18 18 hour Yes stuttering - ischemic 288 388 8.7 liver 3 spleen 10 chronic phase
64 32 4 days Yes stuttering - ischemic 540 210 12.4 liver 3 spleen 15 chronic phase
65 24 14 hours Yes stuttering - ischemic 177.15 N/A 10.3 N/A chronic phase
65 29 6 hours Yes stuttering - ischemic 402.24 N/A 8.2 N/A chronic phase
56 60 2 weeks splenectomy 16 months before stuttering - ischemic 360 222 N/A had splenomegally, splenectomy chronic phase
66 13 3 days Yes stuttering - ischemic 350 450 8.5 spleen 4cm chronic phase
67 24 prolonged Yes stuttering - ischemic 540 N/A HCT 25% spleen 7 cm chronic phase
51 33 3-5 days Yes stuttering - ischemic 197 350 N/A spleen 14 cm chronic phase
51 27 NA Yes stuttering - ischemic 202 900 N/A spleen 12cm chronic phase
51 33 38 days Yes ischemic - stuttering 240 1150 N/A spleen 19 cm chronic phase
51 28 70 days Yes stuttering - ischemic 186 218 N/A spleen 7 cm chronic phase
51 28 24 hr Yes stuttering - ischemic 500 345 N/A spleen 14 cm chronic phase
68 11 12 hours Yes stuttering - ischemic 290 550 he ma toe re te 22% spleen 4 cm, mild hepatomegally chronic phase
51 23 duration after presentation 36 days Yes ischemic - stuttering 470 180 N/A spleen 14 chronic phase
50 17 24 hours Yes stuttering - ischemic 290 230 hematocrit: 28 per cent; massively enlarged spleen, occupying the entire left half of the abdominal cavity chronic phase
69 7.5 few hours Yes stuttering ischemic 337 N/A 7 splenomegally chronic phase
70 15 2 times resolved spontaneously Yes stuttering - ischemic 480 130 9.4 mildly enlarged on US chronic phase
41 9 sveral days Yes stuttering - ischemic 509 1200 10.1 hepato-splenomegaly chronic phase
41 9 4 days Yes stuttering - ischemic 169 663 10.3 splenomegally chronic phase
71 36 34 hour Yes ischemic - stuttering 65 800 N/A spleen 12cm chronic phase
71 30 4 day Yes ischemic - stuttering 356.4 220 PCV. 22 L/L liver 6cm, spleen 10cm chronic phase
37 29 3 months duration Yes stuttering - ischemic 284 370 10.5 spleen 3 cm chronic phase
72 18 12 days Yes stuttering - ischemic 199 504 HC 17 spleen 18 cm chronic phase
37 26 3 days Yes stuttering - ischemic 292 490 8.9 spleen 15 cm chronic phase
73 12 2 dyas Yes ischemic - stuttering 346 924 9 liver and spleen were enlarged, 5 cm chronic phase
74 22 one month intermittent Yes stuttering-ischem ic 185 N/A 10.7 splenomegaly on US chronic phase
75 19 over 24 hours Yes stuttering ischemic 296 936 9.2 spleen 3 cm , liver 1-2 chronic phase
76 27 9 hours diagnosed 19 y stuttering - ischemic 450.01 509 11.4 splenomegally on US chronic phase
77 18 6hours Yes ischemic stuttering 588 109 7.3 N/A chronic phase
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Most patients had lower hemoglobin (Hb) levels, and in two reviews, the CML patients with priapism had lower Hb levels than their matched CML patients who didn’t have priapism.

Treatment modalities (Table 2) included medications, aspiration, and irrigation to the corpora cavernosa, radiotherapy, leukoreduction, and surgical shunts. Medications were used in 59 CML patients, aspiration of the corpora cavernosa in 49 patients, leukapheresis in 19 patients, radiotherapy in 9 patients, and shunt in 40 patients.

Table 2.

Treament modalites of priapism and the outcome of erectile dysfunction

patient refernce medications for priapism Aspiration and irrigation leukophresis irradiation shunt Best responded to Erectile dysfunction
10 Imatinib No Yes No No leukapheresis No
11 No Yes No No glanocorposal shunt, corporospongiosal shunt shunt N/A
12 No Yes No No Winter’s shunt Winter’s shunt No
13 No Yes Yes No No leukapheresis No
14 Yes Yes Yes No No after leukophresis and medication N/A
15 No Yes No No surgery penis shunts surgery penis shunts N/A
16 No Yes No No Winter’s shunt Winter’s shunt partial response, hydroxyurea after combined complete response Yes
17 Yes Yes No No bilateral T-shunts afte shunt Yes
18 No No No No Winter shunt Winter shunt N/A
19 No Yes No No No cavernosa aspiration, epinephrine irrigation No
20 No Yes No No No cavernosa aspiration and irrigation with epinephrine No
21 No No No No corpus cavernosa-glans shunt corpus cavernosa-glans shunt N/A
22 No Yes No No No Aspiration following intra- cavernosal injection of phenylephrine. N/A
23 No Yes No No No cavernosa aspiration and epinephrine irrigation No
24 hydroxyurea, allopurinol, Cytarabine Yes No No corporoglandular shunting corporoglandular shunting N/A
25 No Yes No No No aspiration and irrigation of the corpora cavernosa N/A
26 imitinib Yes Yes No No Failed aspiration, Leukapheresis ended in, penile prosthesis, Yes
27 allopurinol hydroxyurea Yes Yes No transglandular cavemosum- spongiosum shunt transglandular cavemosum- spongiosum shunt N/A
28 No Yes No No proximal corpora cavernosa-corpus spongiosum shunt surgical proximal corpora cavernosa-corpus spongiosum shunt No
29 No No Yes Yes No improved after 48 hr leukapheresis procedure N/A
30 No Yes No No No aspiration and irrigation with ephedrine Yes
31 hydroxyurea No Yes No No hydroxyurea ,leukopheresis 5 sessions N/A
32 hydroxyurea allopurinol No No No Winter’s shunt shunt, hydroxyurea, allopurinol failed Yes
33 oral pentazocaine, Allopurinol Hydroxyurea, busulpha n No No No No Allopurinol Hydroxyurea N/A
33 No Yes No No No cavernosa aspiration and epinephrine irrigation No
34 Yes No No No aspiration of the corpora cavernosa N/A
25 No No No No Proximal surgical shunt Proximal surgical shunt was performed Yes
36 No Yes No No No cavernosa aspiration and epinephrine N/A
37 Yes No No winter shunt Winter shunting No
37 hydroxyurea Yes No No Al-Ghorab shunt Aspiration Shunt No
37 hydroxyurea Yes No No Al-Ghorab shunt Aspiration Shunt EHS2. Grade 1 developed ED
38 hydroxyurea Yes No No Al-Ghorab shunt Aspiration Shunt No
39 No Yes No No No cavernosal aspiration, irrigation and phenylepinephrin partial response after 5 days improved N/A
40 Hydroxiurea ARA-C No Yes No No Hydroxiurea ARA-C / LeukapheresisOne session per day for 3d /LMWH N/A
41 No Yes No No No corporal aspiration N/A
42 Cyclophosphamide LMWH SC No Yes No No N/A N/A
43 No Yes No No transglanular to corpus cavernosal shunt transglanular to corpus cavernos-al shunt N/A
44 hydroxyurea No yes No transglandular cavernospongiosum shunt transglandular cavernospongiosum shunt surgical curettage of the penis.
44 NA Yes No No winter’s T shunt, N/A N/A
44 NA Yes No No No N/A N/A
45 NA Yes No No winter’s T shunt N/A N/A
46 Vincristine sulfate and prednisone No No Yes No radiation,complete resolution after 6 d N/A
47 imatinib Yes No No No cavernosaaspiration was unsuccessful. Imatinib N/A
48 NA Yes No No Distal corporoglanular shunt N/A N/A
49 analgesics, anxiolytics and steroids Yes No No No bilateral aspiration and irrigation N/A
50 hydroxyurea, allopurinol and intravenous fluids imatinib No Yes No No leukapheresis N/A
51 busulfan No No No No subsided gradually over a two to three week period Yes
51 hyaluronidase No No Yes No NA N/A
51 Busulfan No Yes No No NA No
51 Busulfan, hydroxurea No Yes No sapheno-cavernous bypass. NA Yes
52 Busulfan, hydroxurea No Yes No sapheno-cavernous bypass. NA N/A
52 pain killers NA No No Proximal shunt N/A N/A
52 Cavernosal Pseudo- Ephedrine Inj, pain killers NA No No No N/A N/A
52 pain killers NA No No Proximal shunt N/A N/A
52 Cavernosal Pseudo- Ephedrine Inj, pain killers NA No No No N/A N/A
52 pain killers NA No No No N/A N/A
53 N/A Yes No No No N/A N/A
53 NA NA No No Winter shunt NA Yes
53 NA NA No No Winter shunt NA Yes
53 NA NA No No Winter shunt NA Yes
53 NA NA No No Winter shunt NA Yes
54 NA NA No No Winter shunt NA Yes
54 allopyranol hydroxyurea NA No No cavernosoum- spongiuosum shunt allopyranol hydroxyurea, cavernosoum- spongiuosum shunt No
55 allopyranol hydroxyurea NA No No cavernosoum- spongiuosum shunt allopyranol hydroxyurea, cavernosoum- spongiuosum shunt No
56 myeleran endoxan No No Yes No radiation to the penis N/A
57 NSAIDS and Diethyl Stilbesteol No No No glandulo-cavernosal shunt improved after the shunt Yes
58 No Yes No No No N/A N/A
59 cold compressision, priscoline hydrochloride, diethylstilbestrol No No Yes No radiation therapy the penis N/A
60 benzyle penicilin busulfan, trioxyphenbutazone Yes No No No initial aspiration little response improved after irrigation (lowselys operation ) N/A
61 N/A NA No No NA immediate surgical decompression N/A
62 NO NO NO NO saphenocavernous anastomosis N/A N/A
63 Yes Yes Yes no corpus cavernosa-glans shunt Aspiration and irrigation Yes
64 Busulfan Yes No No No with bothe busulfan, irrigation and aspirartion of the corpora cavernosa Yes
64 busulfan Yes No Yes No improved after aspiration and irrigation Yes
64 No Yes No Yes No after aspiration and irrigation of corpora cavernosa Yes
65 Yes No No No No N/A N/A
65 Yes No No No No N/A N/A
56 hydroxyurea allopurinol Imatinib Yes No No No improved after aspiration Yes
66 allopyranol Yes Yes No Distal shunt r distal distal shunt procedure N/A
67 Myleran intravenous A-139 1, demerol No No No No flaccid after two weeks of medical therapy N/A
51 hydroxyurea No Yes No sapheno-cavernous bypass. after sapheno-cavernous bypass. Yes
51 N/A Yes No No No N/A N/A
51 Busulfan Yes No No No N/A Yes
51 Busulfan Steroids. Anticoagulants. No No No No N/A Yes
51 busulfan No Yes No No N/A Yes
68 allopyranol hydroxyurea No No No No the priapism subsided after 24 hr of starting medicalk treatment no aspiration was done No
51 Busulfan Anticoagulants NA No Yes No no benefit Yes
50 busulfan Yes No No shunt between the right saphenous vein and corpus cavernosus shunt between the right saphenous vein and corpus cavernosus N/A
69 busulfan then, 6- mercaptopurine, busulfan again No No Yes No improbed after radiotheray on the 2 weeks period N/A
70 No No No No No on presentation no priapism was there , two episodes of priapism that resolved, and he was treated three times with metronidazole for presumed balanitis. N/A
41 1 LMWH SC BID for one month , Hydroxyurea Cyclophosphamide No Yes No No N/A N/A
41 1 LMWH SC / hydroxyurea No No No No Hydroxyurea N/A
71 hydroxycarbamide aspiration done No No No hydroxycarbamid aspirin 4 units of PRBC No
71 cyclophosphamide 1g stat, hydroxycarbamide 1g 12 hourly No No No No 20th day of admission with significant healing of the penile shaft ulcers, significant detumescence N/A
37 No No No No No Imatinib developed ED EHS2. Grade 1
72 Yes No No No No By the 4th week of cytoreduction Yes
37 hydroxyurea Yes No No Al-Ghorab shunt Aspiration Shunt developed ED EHS2. Grade 2
73 Yes No No No No terbutaline 0.125 mg subcutaneously N/A
74 Paracetamol Morphine Diazepam, prednisone Terbutaline terbutaline 5.0mg, hydroxyurea No No No Winter shunt Corpora cavernosa aspiration (winter), prednisone 2, Terbutaline terbutaline 5.0mg, tab hydroxyurea N/A
75 No Yes No No No corporal aspiration and phenylephrine irrigation N/A
76 No Yes No No No aspiration followed by intracavernosal injection of 1 dose of phenylephrine No
77 No Yes No No No penile aspiration and irrigation N/A
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Discussion

CML is a myeloproliferative neoplasm (MPN) characterized by the uncontrolled production of mature granulocytes. The three stages of the CML are the blast phase, accelerated phase, and chronic phase. Most of the patients are diagnosed incidentally with an elevated white blood cell count on the CBC during their chronic asymptomatic period. Unfortunately, in most cases, the diagnosis of CML is reached late, as it has a large variety of vague clinical manifestations that may include lymphadenopathy, fatigue, hepatosplenomegaly, weight loss, bleeding tendency, and thromboembolic phenomena due to hyperleukostasis (78).

The mechanism of priapism in leukemia is believed to be related to blood sludging with white blood cells (6, 79). Severe anemia implies tissue hypoxia, which may interfere with the NO cGMP balance and precipitate the occlusion. Repeated priapism (stuttering) episodes can lead to prolonged ischemia and tissue damage (80). Most patients with priapism had lower Hb levels compared to their matched CML patients without priapism (78,81).

Although anemia may be an essential factor in priapism’s pathogenesis, it is not clear if blood transfusion is useful to alleviate an acute priapic attack, or it may worsen the condition. Blood exchange transfusion for treating patients with SCD and major priapism has been shown to be efficacious and safe.

WHO defines CML’s blast phase as more than 20% blasts (large cells) in bone marrow or peripheral smear (82). Although this could lead to more stasis, unexpectedly, priapism is not more common in the blast crisis phase and accelerated phase (only 5/102 patients). The majority of priapism cases (n = 97/102) occurred during the chronic phase.

In the era before the introduction of tyrosine kinase inhibitors, the chronic phase of CML accounted for 85% of CML presentation at the time of diagnosis (5). Most patients were below the age of 40 years, with a mean of 27.4 years. This means that it is more common in younger patients with CML patients. The peak age of priapism in adults (without CML) is between 20-50 years (83).

Thrombocytosis, with platelets (PLT) count above 600 x10 9/L is seen up to 30% of CML patients (5,84). The high mean PLT count may have some impact on the occurrence of priapism in CML and/or may influence the type of priapism (stuttering versus ischemic) rather than its occurrence. In CML patients with stuttering type priapism, the PLT count was lower (506.8 x10 9/L) compared to those with the ischemic type (609 x109/L). Moreover, there was no significant difference in the PLT count between CML patients with and without priapism (78).

In essential thrombocythemia (ET), another form of MPN with extremely high PLT counts, priapism was much less reported compared to CML (85). The few reports might reflect the minimal role of PLT in occluding the penile circulation compared to WBC.

The enlarged spleen indicates an advanced CML stage, which supports the late presentation. Moreover, splenomegaly is of prognostic importance; massive splenomegaly indicates poor prognosis and increased risk of dying due to CML (86). In this review, splenomegaly was reported in 80/102 (78.4%) patients and hepatomegaly in 31 patients, but organomegaly was not addressed in 20 patients (Table 3). Splenomegaly was seen in 28/31 (90%) with the stuttering type and in 50/70 (73.2%) with the ischemic type.

Table 3.

Characteristics of stuttering and ischemic priapism in patients with chronic myeloid leukemia (CML)

Priapism type Stuttering (n=32) Ischemic (n=70)
Mean time to presentation 220.9 hours (n. 20 ) 77.8 hours
Mean Age (year) 23.78 27.93
Mean WBC 314.9 x109/L 320.49 x109/L
Mean PLT 506.8 x109 /L 609 x109/L
Mean Hb 9.6 g/dl 8.78 g/dl
CML phases All chronic phase 1 accelerated phase
4 blast phase
67 chronic phase
Erectile dysfunction 16 not addressed
13 had erectile dysfunction
3 no erectile dysfunction		 41 not addressed
16 had erectile dysfunction
14 no erectile dysfunction
Splenomegaly (n) 29 51
Hepatomegaly (n) 9 22
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Ethnicity might have a role in the predisposition to priapism as 57% of the CML patients with priapism were Asian. Most of these reports came from India. This could be due to genetic susceptibility or difficulty accessing health care for the nonspecific symptoms of CML until WBC reached high levels, causing vascular stasis and priapism. Also, priapism occurred in patients with a problem with compliance or stopped medications (30, 46,76). Surprisingly, priapism developed after starting cytoreductive therapy in 3 patients (32, 49, 50). It is hard to conclude that initiating cytoreductive therapy will raise the risk of priapism.

Priapism is a urological emergency, which must be treated early to prevent erectile dysfunction. It is predicted that if priapism lasts more than 24 hours, the risk of permanent erectile dysfunction is more than 90% (87). Therefore, a rapid and expert reversal of the priapism is highly required. The mean time that a CML patient with priapism sought medical advice was 78.28 hours (n=76 ), which carried a high risk for developing permanent erectile dysfunction (88). However, despite this delayed presentation to medical attention, the reported erectile dysfunction was not high. The erectile dysfunction after the episode(s) of priapism in CML patients was reported in 29/102 (28.15%) and did not occur in 17/28 (60.7%). Probably, the lack of information and methods used to assess the erectile function in CML patients may explain the low reported percentage of erectile dysfunction in CML patients.

Over the past decade, we have witnessed significant advances in knowledge of CML’s biology and treatment. Imatinib is a first-line tyrosine kinase inhibitor for treating CML and has dramatically improved the prognosis of this disease. Chang et al. (89) have shown that Imatinib crosses the blood-testis barrier and reduces sperm density, sperm count, sperm survival rates, and sperm activity in CML patients in the chronic phase. But did not affect the structure of reproductive organs or sex hormone levels.

Forty patients needed a surgical shunt to relieve priapism, 5 of them had a partial response and continued chemotherapy to control the priapism. Forty-nine patients responded to aspiration and irrigation, and 2 of them required chemotherapy to control priapism due to incomplete response. Irradiation to the penis and spleen was used in 9 patients; leukapheresis was used in 19 patients, 6 of them required surgical shunts ( 43, 51, 63, 66).

Medications alone were used to treat priapism in 14 patients. However, reversing priapism using medications required longer duration compared to other modalities (mean duration in 7 patients was 14.4 days). Medications used included: hydroxyurea (n= 48), cyclophosphamide (n=3), busulfan (n=15), terbutalin (n=1), prednisolone (n=2), vincristine (n=1), priscoline hydrochloride (n=1), hyaluronidase injection (n=1), imatinib (n=5), cytarabine (n=1), diethylstilbestrol (n=2), cytarabine (n=2), low-molecular-weight heparin (n=3), anticoagulation (n=2), opioids (n= 1), blood transfusions (n=2).

The response to systemic therapy alone (medication) is usually prolonged and may represent the natural history of ischemic priapism rather than the effect of the medications (90). The American Urology Association (90) recommended an early treatment in a step-wise fashion starting with therapeutic aspiration (with or without irrigation) or intra-cavernous injection of sympathomimetics.

For CML patients, two points shall be considered. First, the late presentation to medical attention, which means that the slowly acting medications are less likely to be effective alone. In contrast, urological aspiration and irrigation within the first 24 hours decrease the risk of erectile dysfunction (91). Therefore, it is reasonable to start with aspiration and irrigation not to rely on oral medication for the treatment of CML alone. Secondly, treating the underlying mechanism of increased WBC count is needed to control and prevent priapism recurrence. Leukapheresis was used as a modality to treat both the high WBC of CML and priapism. The pitfalls of leukapheresis are that it is not available everywhere, is costly, and may require several sessions before a significant reduction in WBC count, which may take days. Besides, there is no clear-cut WBC value below which priapism is anticipated to be controlled. The mean WBC count after which priapism was controlled, is 22 x10 9/L. However, other patients were controlled only with WBC count between 3-10 x10 9/L. It remains an option for patients who failed aspiration and refused surgical shunt.

Similarly, the effects of irradiation were not rapid (6 days, 2 weeks, and 19 days), as documented in a few patients (45,58,69).

Four major types of surgical shunts are used for the treatment of priapism. These include percutaneous distal shunts, open distal shunts, open proximal shunts, and vein anastomoses/shunts (92). The goal of surgery is to create a channel or fistula that allows the deoxygenated blood to drain from the corpora cavernosa. For all shunt procedures, the patient should receive preventive perioperative antibiotics.

Guidelines advocate for an aggressive approach in treating patients with refractory priapism by proceeding in a serial fashion from distal to proximal shunts to vein shunting as quickly and safely as possible to achieve penile flaccidity (92).

A delayed penile implant was used in one patient. It is generally used for patients who developed erectile dysfunction; however, it can be used acutely to control priapism and prevent fibrous tissue formation (93). The erectile dysfunction occurs more frequently following proximal or vein shunts compared to the distal shunts. However, it is difficult to attribute the erectile dysfunction to the shunt operation as patients had received different modalities of treatment and had different duration before seeking medical attention (89).

Conclusion

Priapism is a rare complication of CML. It is mostly seen at the first presentation of the disease and much less during the start of treatment. It may occur after stopping the medication or post-splenectomy. Late presentation negatively affects the response to treatment as well as erectile function. Therefore, physicians must interfere early and follow a timely plan and shall follow the patients closely for developing erectile dysfunction. Conservative and medical therapy without urological intervention is less likely to be sufficient. Starting treatment of CML to decrease the high WBC count might accelerate the resolution of the priapism and sometimes is needed for a complete resolution.

Conflicts of interest:

Each author declares that he or she has no commercial associations (e.g. consultancies, stock ownership, equity interest, patent/licensing arrangement etc.) that might pose a conflict of interest in connection with the submitted article.

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