PRIAPISM AND CHRONIC MYELOGENOUS LEUKEMIA.

Wajih Ullah M, Rehman A, Cheeti A, Siddiq W, Latif WA, Prasai K and Bai Joti. 1. Card iology, Mayo Clin ic, Rochester, USA, 802 1st ST SW, Rochester, MN, USA. 2. Observer In Internal Medicine, Bay lor Saint Luke's Medical Center, Houston, USA. 3. Internal Medicine, Kamineni Institute of Medical Sciences, Narketpally, IND. 4. Internal Medicine, Harvard Medical College/Beth Israel Deaconess Medical Center, Boston, USA 5. Internal Medicine, Fat ima Jinnah Medical University, High Point, USA. 6. Oncology, Mayo Clinic, Rochester, MN, USA. 7. Internal Medicine, Liaquat University of Medical and Health Sciences Hospital Jamshoro Sindh Pakistan., Jamshoro, PAK. ...................................................................................................................... Manuscript Info Abstract ......................... ........................................................................ Manuscript History

Chronic myelo id leu kemia (CM L) is a cancer of the wh ite blood cells and the bone marrow. It is characterized by an increased and unregulated growth of myeloid cells in the bone marrow and accumulat ion of these cells in the blood. There are three stages of CM L, and most patients are diagnosed incidentally with an elevated white blood cell count seen on the complete blood count (CBC). Occasionally, CM L patients present with upper quadrant pain due to hepatosplenomegaly. An enlarged spleen may put pressure on the stomach, causing loss of appetite and weight loss. CML patients may also present with fever, n ight sweats, bleeding, or petechiae. An init ial presentation of the CML patient with priapism is very unusual and rare. In this case report, we are documenting a case of a 22 -year-old man who presented with a persistent painful erection of the penis for four days. After emergency treatment for the priapism, the patient underwent multip le investigations including blood morphology, bone marrow aspiration, and BCR-ABL molecu lar testing, which confirmed the diagnosis of CML. The purpose of this study is to highlight the rare presentation of CM L and the importance of diagnosing the underlying cause responsible for the acute presentation. 145 bleeding, purpura, splenomegaly, leukocytosis, anemia, and thrombocytosis [1]. Less commonly, CM L patients can present (mostly in chronic phase) with priapism and incidence of priapism in adult leukemic patients is about 1-5%. In the pediatric population, it is even rarer [2]. The following case illustrates priapism as an initial presenting symptom of an undiagnosed CML patient.
Case Presentation:-An otherwise healthy 22-year-old man presented to the emergency department with persistent, and painful penile erection for four days accompanied by bleeding during micturit ion. There was no history of trauma, fever, night sweats, joint pain or rash. He did not have any antecedent infection. The patient's past medical, surgical and family history was unremarkable. He had no known allergic reaction to food or prescription medications. He did not use any illicit drugs.
His vital signs on examination were (1) Temperature: afebrile (2) Blood Pressure: 135/85 mmHg (3) Respiratory Rate: 21 breaths/min (4)Heart Rate: 108 beats/min. Pu lse oximetry showed 98% o xygen saturation in room air. He was alert and oriented to time, place, and person. Physical examination of the patient revealed a palpable liver 5 cm below the right costal marg in, and the spleen was palpable 6 cm below the left costal margin. The penis was erect, firm, and tender with superficial venous engorgement. The rest of the systemic examination was unremarkab le.
The patient was scheduled for the aspiration of b lood and irrigation of the corpora cavernosa a t the emergency department for his painful, sustained erection. The patient felt relief after these procedures, and he was admitted to the Hematology department for further investigations of his hyperleukocytosis. The peripheral blood smear of the patient showed myeloid hyperplasia with neutrophilia, myelocytes, and metamyelocytes. The patient was scheduled for a bone marro w biopsy. The biopsy showed myeloid hyperplasia with the predominance of neutrophils and metamyelocytes. The patient's peripheral blood smear and bone marrow findings raised the suspicion of CML. Detection of BCR-A BL confirmed the diagnosis of Chronic phase CML, with priapism as the initial presentation. The patient was started on hydroxyurea 1.5 grams daily and one vial of interferon alfa -2a subcutaneously daily. Allopurinol 300 mg daily with adequate hydration was also started. Before discharge, his white blood cells dropped to 76000/cu mm and hemoglobin raised to 10 mg/dl. In subsequent visits, recurrent priapism did not happen, and his leukemia was in remission.

Discussion:-
Priapism is defined as a persistent penile erection that continues at least four hours unrelated to sexual stimu lation [3]. It is a urological emergency, which must be treated early to prevent erectile dysfunction. Priap ism is a rare condition on its own with an incidence of 1.5:100,000 person -year [4]. In men, 20% of the cases are caused by the hematological conditions such as sickle cell anemia, chronic myelogenous leukemia, chronic ly mphocytic leukemia, and acute lymphoblastic leukemia [2]. In adult leukemic patients, the incidence of priapis m is 1-5%, and in the pediatric population, it is even rarer. Ho wever, as an initial presenting feature of CM L, priapis m is seen in 1-2% of cases only.
In patients with leukemia, priapism mainly occurs due to hyperleukocytosis which results in blockade of the veins [3]. Two mechanis ms are proposed for the development of priapis m in leu kemic patients. In the first theory, sludging of leukemic cells in the corpora cavernosa and the dorsal penile vein. The second mechanism proposes that the sacral nerves and the central nervous system beco me infiltrated with leukemic cells. In this study, we report a case of a 22-year-old man who came to the emergency department with an initial presentation of priapism for CM L. It is a rare clin ical presenting feature and only seen in 1-2% of cases.
There are two types (high flow and low flow) of priapis m, and the management varies with the type. Low flow (ischemic) priapis m is mostly seen in CML due to occlusion of corpora cavernosa, and therefore it is considered of the urological emergency requiring urgent intervention. Because priapis m in CM L is a rare occurrence, there is no standard treatment according to the guidelines. However, the American Uro logical Association (AUA) has published some guidelines for the treatment of ischemic priap ism. AUA reco mmends treating the ischemic priap ism 146 and then doing investigations to find out the real cause of the priapis m [5]. Once the cause is identified, th en the next step is to treat the condition. According to the AUA guidelines, it is required to administer intra -cavernosal treatment concurrently.
In our case, the patient felt relief after the cavernosal aspiration and epinephrine irrigat ion. After that , the patient underwent mult iple investigations and was diagnosed with chronic phase CML. The patient was started on med ications for CM L and also given allopurinol 300 milligrams and hydro xyurea 2 grams daily to ensure cytoreduction. The primary objective of this study is to highlight the importance of principal diagnosis and management of priap ism, as there is a very high risk for impotence follo wing this comp licat ion. Apart fro m the initial management of the priap ism, the need for further investigations cannot be overemphasized as it can lead to the proper diagnosis of the underlying cause.

Conclusions:-
CM L is a cancer of the white blood cells and bone marrow. It is usually diagnosed incidentally, in its chronic asymptomatic phase. The most common symptoms in its presentation are fatigue, weight loss, abdominal fullness, bleeding, purpura, splenomegaly, leu kocytosis, anemia, and thro mbocytosis. However, in rare cases (1-2% of adults) it can present with priapis m as its in itial presenting comp laint. In th is study, we would like to highlight the importance of principal d iagnosis and management of priapis m, as delayed treatment of priapis m has a high risk of impotence. Also, we would like to emphasize the need to evaluate the patient further once priapism ha s been managed, to check for the underlying cause of the priapism and appropriately managing the condition to prevent relapses of the priapism.