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 Table of Contents  
Year : 2022  |  Volume : 12  |  Issue : 2  |  Page : 79-84

Priapism as the initial presentation in chronic myeloid leukemia is a red flag sign: A case report

Department of General Medicine, Assam Medical College and Hospital, Dibrugarh, Assam, India

Date of Submission10-Aug-2022
Date of Acceptance16-Aug-2022
Date of Web Publication23-Nov-2022

Correspondence Address:
Dr. Aritra Saha
Department of General Medicine, Assam Medical College and Hospital, Dibrugarh, Assam
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajoim.ajoim_13_22

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Chronic myeloid leukemia (CML) is a myeloproliferative disorder of the hemopoietic stem cells, which may occasionally present with symptoms of leukostasis secondary to hyperleukocytosis, which includes headache, priapism, and dreaded ones such as stroke and myocardial infarction. This is a case of a 23-year-old man who presented with priapism and was subsequently found to have CML. The patient later developed stroke and succumbed to it. This case demonstrates that the importance of treating CML patients presenting with signs of hyperleukocytosis promptly and cautiously to prevent the impending grievous complications.

Keywords: CML, CVA, hyperleukostasis, priapism

How to cite this article:
Saha A, Visvesvaran S, Narzari S, Das K, Pegu AK, Dhanowar RK. Priapism as the initial presentation in chronic myeloid leukemia is a red flag sign: A case report. Assam J Intern Med 2022;12:79-84

How to cite this URL:
Saha A, Visvesvaran S, Narzari S, Das K, Pegu AK, Dhanowar RK. Priapism as the initial presentation in chronic myeloid leukemia is a red flag sign: A case report. Assam J Intern Med [serial online] 2022 [cited 2023 Mar 22];12:79-84. Available from: http://www.ajimedicine.com/text.asp?2022/12/2/79/361825

  Background Top

Chronic myeloid leukemia (CML) is a myeloproliferative disorder of the hemopoietic stem cells carrying BCR-ABL gene in 90% of cases and is responsible for augmented tyrosine-kinase activity causing unregulated cell proliferation.[1] Occasionally, patients may have hyperleukocytosis (total leukocyte count [TLC] > 100 × 109/L) associated symptoms of hyperviscosity, which includes headache, priapism, myocardial infarction, stroke, retinal hemorrhage, etc.[2] Leukostasis as a result of hyperleukocytosis occurs in around 12% of adult CML patients and prompt treatment for leukostasis includes adequate hydration, treatment with tyrosine kinase inhibitor [TKI], and high-dose hydroxyurea and leukapheresis.[2]

Here, we present the case of a 23-year-old man presenting with priapism, who after undergoing cavernosal aspiration, was started on hydroxyurea and imatinib, but leukapheresis could not be done because of a lack of that facility, nor we could refer the patients due to COVID-related travel restrictions and financial constraints from the patient’s aspect. The patient later developed stroke and succumbed to the illness within minutes after its onset.

  Case Presentation Top

A 23-year-old man, resident of northern region of the state of Assam, presented to the Department of Casualty of our institute with the chief complaint of persistent penile erection for more than 3 days, associated with generalized weakness. The patient was attended by the doctors of Department of Urology and Department of General Surgery, and General Medicine opinion was taken. The patient did not give a history of any trauma, bleeding diathesis, insect bite, fever, the use of any sexual stimulant or recreational drug, similar history in the past, or family history. The patient had no known comorbidities and any significant past medical history. The patient belonged to lower socioeconomic strata with no known allergic history. The patient was immediately taken up for corpora cavernosal aspiration along with an injection of epinephrine and was also planned for shunt surgery later in the course of disease.

On general examination, the patient was alert, conscious, well-oriented to time, place, and person. The patient had an average built and was in an anxious state of mind. The patient had lymph node enlargement in the inguinal region, multiple in number, measuring approximately 1.5 cm, mobile, nontender, and nonmatted. Remaining general examination findings were insignificant.

Per-abdominal examination revealed massive splenomegaly along with palpable liver. Remaining systemic examination findings were within normal limits.

Routine investigations, that included complete blood count (CBC) and PBS, was suggestive of CML (TLC: 7.21 × 105/cumm; differential leukocyte count [DLC]: neutrophil = 34%, lymphocyte = 02%, monocyte = 01%, eosinophil = 03%, basophil = 04%, myeloblast = 03%, myelocyte = 26%, promyelocyte = 02%, metamyelocyte = 10%; peripheral blood smear [PBS]: hyperleukocytosis with a plenty of immature myeloid cells and basophils) [Figure 1], ultra-sonogram [USG]: penile Doppler revealed absent blood flow in the bilateral cavernosal arteries, suggestive of ischemic priapism [Figure 2], and USG-whole abdomen corroborated the examination finding of splenomegaly (spleen size = 20.7 cm with mild hepatic enlargement).
Figure 1: Peripheral blood smear showing immature myeloid cells

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Figure 2: Penile Doppler: Absent blood flow in bilateral cavernosal arteries

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Bone marrow study and BCR-ABL study was done. Bone marrow aspiration was suggestive of myeloproliferative neoplasm in favor of CML in chronic phase (CP) [Figure 3] (myelocyte: 30%; blast: 02%; basophil: 06%), and the patient tested positive for BCR-ABL with B3A2 mutation, a week later. Additional investigations were done to rule out the other causes of priapism including hemoglobin typing and blood glucose levels, both were within normal limits. As the patient started complaining of blurring of vision, a fundoscopic examination was done, which revealed flame shaped retinal haemorrhage bilaterally, suggestive of retinopathy [Figure 4]. Routine examination of urine, which was within normal limit.
Figure 3: Bone marrow aspiration: CML in CP

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Figure 4: Fundoscopy: Flame-shaped hemorrhage in bilateral fundus

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After being diagnosed with CML, the patient was shifted to the Department of General Medicine. In order to relieve the priapism component, the patient was also planned for shunt surgery, but the patient’s party refused it. Because the other causes of ischemic priapism were ruled out, we started treating leukostasis as a result of CML conservatively with tablet hydroxyurea 2 g per day in divided doses and tablet imatinib 400 mg per day. Total leukocyte count and serum uric acid levels were repeated daily to see the response and occurrence of tumor lysis syndrome. As the uric acid levels was on a rising trend (9.70 mg/dL), tablet febuxostat 40 mg per day was started. The total counts started reducing to 6.3 × 105/cumm on day 2 and 6 × 105/cumm on day 3. The patient felt symptomatically better in terms of pain and the extent of penile erection.

Leukapheresis was considered as a treatment option, but because of the lack of the facility and inability of the patient’s party to take the patient to a higher center due to COVID-19–related travel restrictions, the treatment was continued conservatively in our setup.

However, on day 4, the patient developed sudden onset altered sensorium in the absence of fever, associated with slurring of speech, left-sided hemiparesis, and extensor plantar response on the left side. Pupil was mid dilated and nonreactive to light with the angle of mouth deviated to the right side. The patient was taken up for non enhanced computed tomography (NECT) brain and then transferred to intensive care unit. NECT brain revealed intraparenchymal bleed in the right basal ganglia region [Figure 5]. Osmotherapy with 20% mannitol infusion was started and the patient was intubated for airway protection; however the patient succumbed to the illness soon after.
Figure 5: NECT brain: Intraparenchymal hemorrhage in the right basal ganglia region

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  Discussion Top

CML is a chronic myeloproliferative neoplasm, which results from the clonal expansion of pluripotent hematopoietic stem cells that is produced by a reciprocal chromosomal translocation between the ABL oncogene on chromosome 9 and the BCR gene on chromosome 22.[3],[4],[5],[6],[7] Most common clinical manifestations of CML are anorexia, weight loss, malaise, sweating, and bleeding episodes due to platelet dysfunction, but patients may also present with leukostatic complications of hyperleukocytosis such as thromboembolic phenomena, hearing loss, or priapism.[8],[9],[10],[11]

Hyperleukocytosis is defined as circulating blast cell count or white blood corpuscle (WBC) count exceeding 50 to 100 × 109/L. Hyperleukocytosis may be seen at the presentation of acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, or chronic lymphocytic leukemia.[11],[12],[13],[14] Hyperleukocytosis leads to leukostasis that may be complicated by hearing loss, papilledema, cerebellar dysfunction, impairment of memory function, intracranial hemorrhage, respiratory depression or failure, acute renal failure, and priapism.[10],[11],[14],[15],[16],[17] Treatment options of hyperleukocytosis and leukostasis consist of cytoreductive therapy with hydroxyurea, cyclophosphamide or cytosine arabinoside, and therapeutic leukapheresis. Hyperleukocytosis in CML causes the formation of leukocyte aggregates or leukostatis, which leads to thrombi formation in small blood vessels that ultimately cause vascular obstruction.[8],[9]

Priapism is sustained penile erection in the absence of sexual activity, generally defined as an unwanted erection lasting more than 4 h. Priapism is a urological emergency, which needs early treatment to prevent erectile dysfunction, and if priapism lasts more than 24 h, the risk of permanent erectile dysfunction is more than 90%.[18] Although it may occur at any age, it has a bimodal age distribution, with a peak in the early childhood (5–10 years), owing to sickle cell disease, and a secondary peak that occurs in patients with active sexual activity, between the age of 20–50 years old.[19] Idiopathic priapism is the most common one (64%), whereas approximately 20% are related to hematological disorders.[2],[19]

The following five pathophysiological mechanisms of priapism have been postulated in CML: (1) venous congestion of the corpora cavernosa due to mechanical pressure exerted by the enlarged spleen on the abdominal veins, (2) sludging of leukemia cells in the corpora cavernosa and dorsal veins of the penis, (3) infiltration of leukemia cells in sacral nerves, (4) central nervous system infiltration by leukemia, and (5) hyperleukocytosis causing the elevation of whole-blood viscosity and this ultimately causing complication due to vascular obstruction.[8],[20] Priapism can be diagnosed by detailed history, through physical examination, appropriate laboratory investigations including CBC, differential cell count, and peripheral blood film, cavernosal blood gas analysis, and color duplex ultrasonography.[21] The American Urology Association recommended an early treatment other than drug therapy in a step-wise fashion starting with therapeutic aspiration (with or without irrigation) or intracavernous injection of sympathomimetics.[22] New therapies include (1) cytoreductive therapies with chemotherapeutic agents such as hydroxyurea, (2) leukapheresis in case of hyperleukocytosis, (3) allopurinol, hydration, and symptomatic treatment, and (4) early initiation of TKI therapy with agents such as imatinib.[13],[23]

A stroke or cerebrovascular accident (CVA) is a medical emergency and is characterized by acute compromise of the cerebral vasculature or perfusion, which is further classified as ischemic and hemorrhagic, the former being more common otherwise, but the hemorrhagic variant is more common in the northeastern part of the country.[24],[25] Stroke in a CML patient is relatively less common but is more commonly seen with blast crisis than chronic phase. Exact mechanism is not completely understood, but coagulopathy in blast crisis and hyperleukocytosis in chronic phase is implicated for the occurrence of intracranial hemorrhage in CML patients.[26] As of date, only eight patients were reported to develop intracranial hemorrhage in chronic phase, among which only one patient presented with spontaneous intracranial hemorrhage as the initial feature.[26] While the management of intracranial haemorrhage (ICH) secondary to coagulopathy is mostly medical, a bleed within 1 cm from the cortical surface may be surgically evacuated if associated with midline shift or mass effect, as per American Heart Association.[27]

Our case is unique and interesting because our patient was the youngest among all the other reported CML patients in chronic phase who developed intracranial bleed spontaneously, and to the best of our knowledge, there are no known CML patients in chronic phase to have presented with priapism as the initial presenting feature and later developed spontaneous intracranial bleed.

  Conclusion Top

Initial presenting feature of CML may vary and is often vague, but in certain instances, patients may present with symptoms of leukostasis as a result of hyperleukocytosis, which includes headache, visual disturbance, priapism, and, in unfortunate circumstances, may present with myocardial ischemia and CVA; therefore similar symptoms in CML patients should be considered as a red flag sign and must be treated promptly with modalities such as cytoreductive therapy, leukapheresis, early initiation of TKI therapy, and supportive management.

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Conflicts of interest

There are no conflicts of interest.

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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