|Year : 2017 | Volume
| Issue : 2 | Page : 68-72
Chronic myeloid leukemia with an initial presentation as ischemic priapism: A case report and review of literature
Abubakar U Musa1, Muhammad A Ndakotsu1, Sani B Abubakar1, Peter N Agwu2
1 Department of Hematology, Usmanu Danfodiyo University Teaching Hospital (UDUTH), Sokoto, Nigeria
2 Department of Surgery, Usmanu Danfodiyo University Teaching Hospital (UDUTH), Sokoto, Nigeria
|Date of Web Publication||4-Apr-2018|
Dr. Abubakar U Musa
Department of Hematology, Usmanu Danfodiyo University Teaching Hospital (UDUTH), Sokoto
Source of Support: None, Conflict of Interest: None
Chronic myeloid leukemia (CML) is one of the myeloproliferative disorders accounting for 15–20% of adult leukemia. Priapism is a rare initial presentation of CML occurring in 1–2% of cases. It is a urologic emergency requiring urgent multidisciplinary management as delay in initiating treatment may lead to erectile dysfunction. Combined treatment modalities are usually employed in its management including surgery, chemotherapy, therapeutic leukapheresis, and local intracavernous therapy. We report an 18-year-old male who presented with a 12-day history of priapism. Further evaluation with peripheral blood film revealed CML in chronic phase with ischemic prolonged priapism. At presentation patient refused consent for surgical intervention and subsequently had cytoreductive therapy with oral hydroxyurea. He achieved complete detumescence after 4 weeks of cytoreduction and by the 5th month during follow-up, he developed erectile dysfunction.
Keywords: Chronic myeloid leukemia, erectile dysfunction, ischemic priapism, surgical intervention
|How to cite this article:|
Musa AU, Ndakotsu MA, Abubakar SB, Agwu PN. Chronic myeloid leukemia with an initial presentation as ischemic priapism: A case report and review of literature. Arch Int Surg 2017;7:68-72
|How to cite this URL:|
Musa AU, Ndakotsu MA, Abubakar SB, Agwu PN. Chronic myeloid leukemia with an initial presentation as ischemic priapism: A case report and review of literature. Arch Int Surg [serial online] 2017 [cited 2018 Nov 19];7:68-72. Available from: http://www.archintsurg.org/text.asp?2017/7/2/68/229186
| Introduction|| |
Chronic myeloid leukemia (CML) is one of the myeloproliferative disorders and is a malignant clonal disease of the hematopoietic stem cells resulting in increased myeloid cells, platelets, and erythroid cells., With the worldwide annual incidence of 1–1.5/100,000 population and M:F ratio of 1.8:1, CML accounts for 15–20% of all leukemias in adults. A lower median age of 38 and 67 years has been reported in Nigeria and in the Western World, respectively.,,
Priapism is a rare initial presentation of CML occurring in 1–2% of cases and is of the low-flow (ischemic) veno-occlusive type associated with hyperleukocytosis and leukostasis or hyperviscosity syndrome.,,,, Priapism occurring in CML is a urologic emergency requiring urgent multidisciplinary management as delay in treatment may lead to complications such as erectile dysfunction.,, Modalities for its management include a combination of cytoreductive therapy, therapeutic leukapheresis, and local intracavernous therapy such as aspiration (with or without saline irrigation), intracorporeal injection of sympathomimetics, corpus cavernosal shunting, embolization, or radiotherapy as well as penile prosthetic implant.,,,
This report underscores the need for high index of suspicion for timely diagnosis of CML as a cause of priapism in previously healthy individuals.
| Case Report|| |
The patient was an 18-year-old male student who presented to the emergency unit of UDUTH Sokoto, North-Western Nigeria with a 12-day history of spontaneous onset of prolonged, painful penile erection. No associated history of trauma, sexual arousal/intercourse, use of aphrodisiacs, psychotropic drugs, or local concoctions. He had no history of dysuria or urethral discharge. He has had two similar episodes about a year and 3 months prior to presentation, and each episode lasted less than 24 h and resolved spontaneously. There was no history suggestive of sickle cell disease (SCD) in the family. Four months prior to presentation, he developed blurring of vision and hearing impairment but no bleeding episodes. He first presented at a peripheral hospital where he was admitted and treated for12 days without much improvement, hence his referral to our health facility.
On examination he was anxious looking and in painful distress, pale, anicteric, and without significant peripheral lymphadenopathy or peripheral stigmata of SCD. He had splenomegaly of 12 cm. He had a circumcised male phallus which was erect, turgid with differential warmth and tenderness [Figure 1]. A working diagnosis of ischemic priapism was made by the attending urology team and this was further supported by the finding of absent arterial flow within the cavernosal arteries on penile Doppler ultrasonographic scan (USS) [Figure 2]. Abdomino-pelvic USS revealed splenomegaly of 18 cm. The patient was scheduled for emergency surgical intervention (penile irrigation ± shunting) but informed consent was denied by both the patient and his father on account of fear of impotence as a possible complication.
|Figure 1: Priapism with the patient's penile shaft appearing erect, dark, and turgid|
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|Figure 2: Penile Doppler USS of the patient showing absence of blood flow within the cavernosal arteries consistent with ischemic priapism|
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His hemogram showed a low hematocrit of 17%, platelet count of 504 × 109 l -1 and a high white blood cell (WBC) count of 199 × 109 l -1. The peripheral blood film revealed normocytic normochromic red cells and a complete spectrum of the myeloid series with the following differential counts: myeloblasts 3%, promyelocytes 4%, myelocytes 40%, metamyelocytes 15%, band neutrophils 3%, segmented neutrophils 30%, eosinophils 3%, and lymphocytes 2%; a picture in keeping with CML in chronic phase [Figure 3].
|Figure 3: Peripheral blood smear of the patient showing neutrophilic leukocytosis with complete spectrum of the myeloid series consistent with chronic phase CML|
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Liver and renal function tests as well as uric acid, Ca 2+, Mg 2+, and PO42- were within normal limits, while urinalysis revealed traces of protein and red cells.
Patient was immediately commenced on intravenous hydration with 5% dextrose saline, allopurinol, paludrine, and dihydrocodeine. Cytoreduction with only oral hydroxyurea at a dose of 75 mg/kg/24 h amounting to 1 g 6-hourly was commenced, as there were no facilities for leukapheresis at our center. By the 10th day, priapism persisted splenomegaly reduced to 5 cm and WBC count reduced to 19.2 × 109 l -1, while the hematocrit rose to 19%.
Due to unavailability of facilities for molecular analysis at our center, patient's blood sample could not be analyzed promptly for BCR-ABL detection. Furthermore, the patient could not readily afford the cost of molecular analysis at a private laboratory (Safety Molecular Pathology Laboratory) in Enugu until after about 3 months when the hospital's Patients' Welfare Committee provided him with financial support. The molecular analysis revealed a BCR-ABL1 major (e14a2) transcript type and consequently, the patient was counselled on accessing the free Gleevec therapy at Obafemi Awolowo University Teaching Hospital (OAUTH) Ile-Ife. However, due to financial constrain, he could not afford traveling to OAUTH Ile-Ife and was thus continued on cytoreduction using oral hydroxyurea pending on when he is able to access Gleevec therapy.
By the 4th week of cytoreduction, the patient achieved detumescence and had no clinical splenomegaly. His hemogram normalized with WBC count of 6.7 × 109 l -1, platelet count of 140 × 109 l -1, and a hematocrit of 45.2%. He was consequently discharged on maintenance dose of hydroxyurea at 1.5 g daily and paludrine. He was also followed-up at both the hematology and urology outpatients' clinics.
About 5 months after the initial presentation, while still on oral hydroxyurea, he complained of inability to achieve and maintain tumescence and was subsequently managed for erectile dysfunction by the urology team.
Thereafter, the patient defaulted from follow-up for about 4 months, then he represented with 2-week history of fever, vomiting, diarrhea, and pedal swelling. He was immediately commenced on parenteral ciprofloxacin and metronidazole and specimens were taken for septic workup. Laboratory evaluation revealed neutropenia, neutrophilic toxic granulations, markedly elevated levels of urea, and creatinine, while blood stool and urine culture yielded no growth. He was subsequently managed as a case of acute kidney injury resulting from sepsis alongside the Nephrologists and Clinical Microbiologists. His antibiotic regimen was later on adjusted by the Clinical Microbilogists to include parenteral ceftazidime. In the course of his management, he developed uremic encephalopathy and had two sessions of dialysis before his eventual demise.
| Discussion|| |
Priapism is a persistent penile erection that lasts beyond 4 h, and/or is unrelated to sexual stimulation. It is rare in occurrence with an incidence of 1.5 cases per 100,000 person-years. 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. Priapism is seen in about 1–5% of male patients with all types of leukemia. Though CML accounts for half of the cases with leukemic priapism, it is rare for priapism to be the initial presentation of CML as this occurs in only 1–2% of cases.
In CML, hyperleukocytosis underlies the development of ischemic priapism via aggregation of the leukemic cells in the corpora cavernosa and dorsal veins of the penis as well as infiltration of the sacral veins or the central nervous system by the leukemic cells. Venous congestion of the corpora cavernosa due to mechanical pressure on the abdominal veins by the splenomegaly has also been recognized as a contributing event.
Vital in the management of priapism is the determination of it being either ischemic (low flow) or nonischemic (high flow). The ischemic priapism is the most common type, usually painful and associated with hematological disorders, tumor infiltration, and use of aphrodisiacs or psychotropic drugs. It is a form of compartment syndrome with reduced venous outflow leading to stasis, acidosis, and hypoxia. Aside the usual corpora cavernosal rigidity, physical examination may disclose features of hematological disorders, malignancy, or other underlying disorders. Penile blood gases analysis usually depicts hypoxia, while penile Doppler USS findings are those of little or no arterial flow within the cavernosal arteries. It is a urologic emergency, as if untreated within 24–48 h, irreversible changes and fibrosis may develop and lead to future stuttering priapism or erectile dysfunction.
The nonischemic priapism develops from trauma to the penis or perineum, usually painless and associated with less rigidity of the corpora cavernosa. The penile blood gases analysis results are similar to those of normal arteries, while penile Doppler USS establishes adequate arterial flow within the cavernosal arteries. The nonischemic priapism is not considered as an emergency as the associated increased arterial blood flow ensures adequate blood oxygenation, thus making irreversible changes and fibrosis unlikely. Hence, nonischemic priapism could be treated as an elective case.
Another form of priapism which may complicate hematological disorders is the stuttering priapism, which is a recurrent form of ischemic priapism where unwanted painful erections occur with periods of detumescence. It is particularly seen in SCD and do not commonly lead to permanent erectile dysfunction. Stuttering priapism is rare in CML patients due to the cytoreduction that rapidly occurs with systemic treatment of CML.
The algorithm for the initial management of priapism in CML provided by Chisick et al. seems to be apt for our resource-constrained setting where CML cases usually present late coupled with dearth of diagnostic and therapeutic facilities for its management. According to this algorithm, splenomegaly in a priapic patient should raise the possibility of CML or a related hematological disorder, and in line with this, urgent full blood count with peripheral blood film should be requested., The outcome of these two initial tests will give a direction to additional testing such as hemoglobin electrophoresis, coagulation screening, or bone marrow studies. Penile blood testing and penile Doppler USS are not considered essential in CML as priapism in CML is already known to be of ischemic type, thus intervention should not be delayed on account of waiting to conduct these investigations.
Upon suspicion of CML, parenteral hydration and allopurinol should be commenced to minimize the risk of tumor lysis syndrome, while urgent consultation with the hematology, urology, apheresis services, and hematopathology, units should be sought.
The American Urologic Association recommends that for ischemic priapism, intracavernous treatment should be administered concurrently with systemic treatment of an underlying disorder, such as CML.,, Thus initiation of cytoreduction with hydroxyurea is advocated, while more specific chemotherapy with a tyrosine kinase inhibitor (TKI) should be introduced as soon as possible. If available, combination of chemotherapy with leukocytopheresis is preferred as a single session of leukocytopheresis can reduce WBC count by about 30–60% in cases of hyperleukocytosis., Though the reported evidence for the efficacy of oral sympathomimetics in CML is weak, their use could be considered, while other measures are being taken because if successful, they provide a simple alternative to more expensive or invasive options.,
A systematic approach is employed with the intracavernous treatment., An initial therapeutic aspiration (with or without heparinized-saline irrigation) is conducted and if priapism persists despite several attempts, intracavernous injection of sympathomimetics is performed. It is advocated that surgical shunts should only be considered when sympathomimetics agents have failed. The corporoglanular is considered the primary choice of shunt procedure due to its ease and fewer complications. Presence of severe distal penile edema and tissue damage may necessitate proximal shunt procedures which are more difficult and more likely to develop complications such as urethral fistula, purulent cavernositis, and erectile dysfunction. Failure of nonoperative or the less invasive shunt procedures should prompt insertion of penile prosthesis before significant intracorporal fibrosis sets in, which may make future surgical interventions unfruitful.
Our reported case depicts a late diagnosis of CML with an initial presentation as ischemic priapism. The patient declined intracavernous surgical intervention, while dearth of facilities prevented prompt institution of leukapheresis and determination of his BCR-ABL status for accessing Gleevec therapy. Consequently, the patient had only cytoreductive therapy with oral hydroxyurea and was able to achieve detumescence only after 4 weeks of therapy. At 5 months of follow-up, he developed erectile dysfunction probably due to the already established intracorporal fibrosis which could have been averted via early diagnosis, prompt intracavernous intervention, and appropriate cytoreductive therapy.
| Conclusion|| |
CML should be considered in the differential diagnosis of priapism as early diagnosis and prompt institution of chemotherapy with intracavernous treatment could avert development of erectile dysfunction.
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Conflicts of interest
There are no conflicts of interest.
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