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Year : 2009  |  Volume : 29  |  Issue : 5  |  Page : 412
 

Priapism as the first manifestation of chronic myeloid leukemia


 Department of Medicine, Hematology Unit, Centre Hospitalier Regional, Beni-Mellal, Morocco

Date of Web Publication22-Aug-2009

Correspondence Address:
llias Tazi
Department of Medicine, Hematology Unit, Centre Hospitalier Regional, Beni-Mellal 23000
Morocco
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DOI: 10.4103/0256-4947.55176

PMID: 19700904



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How to cite this article:
Tazi l. Priapism as the first manifestation of chronic myeloid leukemia. Ann Saudi Med 2009;29:412

How to cite this URL:
Tazi l. Priapism as the first manifestation of chronic myeloid leukemia. Ann Saudi Med [serial online] 2009 [cited 2009 Dec 14];29:412. Available from: /text.asp?2009/29/5/412/55176

To The Editor: A previously healthy 33-year-old Moroccan man was referred to the local hospital for persistent painful erection of the penis that had lasted approximately 22 hours. His penis remained erect, painful, and swollen when he arrived at the emergency department. He denied recent intercourse, trauma, use of illicit drugs, use of medications, and radiation therapy. The patient also denied fever, sweats, and chills. The vital signs revealed a body temperature of 37.4 °C, pulse of 98 beats/minute, and blood pressure of 12/7 mm Hg, and respiration of 22 beats/minute. He was alert and oriented. The physical examination revealed that the spleen was palpable 4 cm below the left costal margin. His conjunctiva was pale but no jaundice. On examination, the penis was erect, firm, and tender with superficial venous engorgement. Urinalysis was normal. Laboratory data showed hemoglobin 10.53 g/dL, hematocrit 24%, white blood count

400 000/mm 3 , and platelets 1 200 000/mm 3 . A peripheral blood smear demonstrated immature leukocytes in various stages of differentiation. Karyotype analysis revealed the Ph1 chromosome and myeloid hyperplasia in the bone marrow. A diagnosis of chronic myeloid leukemia (CML) was made. Treatment of the priapism was initially performed by cavernosa aspiration and epinephrine irrigation at emergency department under the impression of low flow-type priapism. The erection was relieved later by these procedures. For hyperleukocytosis, the patient was admitted to the hematological ward. He was started on hydroxyurea therapy (50 mg/kg/day) associated with intravenous fluid hydration and allopurinol (300 mg/day) for potential tumor lysis syndrome. After 15 days of chemotherapy with hydroxyurea, the patient's leukocyte and platelet counts were 8 000/mm 3 and 240 000/mm 3 , respectively. Recurrent priapism had not happened to him during his admission period. The patient was treated with imatinib mesylate for his CML and continued to report to us without any erectile dysfunction until the date of writing.

Priapism is a rare disease, characterized by prolonged, painful and irreducible erection, not resulting in ejaculation. It is an andrological emergency with a poor prognosis, as the risk of impotence is 50% despite appropriate management. Sickle cell anaemia, chronic myelogenous leukemia, chronic lymphocytic leukemia, and acute lymphoblastic leukemia are hematologic disorders that can be a cause of priapism. [1] In adult leukemic patients, the incidence of priapism is estimated to be approximately 5%. [2] Priapism as a result of hematologic malignancy is most likely caused by venous obstruction from microemboli/thrombi as well as hyperviscosity caused by the increased number of circulating leukocytes in mature and immature forms. [3] In cases of hematologic malignancy, controversy has existed regarding the optimal treatment of leukemic priapism. Earlier series of case reports show successful detumescence with local radiation therapy, open surgical shunting, or combination of the two treatments. [4] More recent literature has focused on the use of cytoreductive modalities such as chemotherapy or combination chemotherapy and leukapheresis. [5] Because of the relatively rare occurrence of leukemic priapism and the small number of case series, there is no standard treatment protocol. Chemotherapy or radiotherapy may first be attempted. If detumescence is not achieved, then surgical shunting should be considered.

 
   References Top

1.Schreibman SM, Gee TS, Grabstald H. Management of priapism in patients with chronic granulocytic leukemia. J Urol. 1974 Jun;111(6):786-8.   Back to cited text no. 1    
2.Alluι López M, Garcνa de Jalón Martνnez A, Pascual Regueiro D, Mallιn Mateo E, Villanueva Benedicto A, Rioja Sanz LA. [Priapism as an initial presentation of chronic myeloid leukaemia]. Actas Urol Esp. 2004 May;28(5):387-9. Spanish.   Back to cited text no. 2    
3.Mulhall JP, Honig SC. Priapism: etiology and management. Acad Emerg Med. 1996 Aug;3(8):810-6.   Back to cited text no. 3    
4.Winter CC, McDowell G. Experience with 105 patients with priapism: update review of all aspects. J Urol. 1988 Nov;140(5):980-3.   Back to cited text no. 4    
5.Kumar L, Sagar TG, Majhi U, Shanta V. Priapism in leukaemia-report of three cases. J Assoc Physicians India. 1990 Mar;38(3):229-30.  Back to cited text no. 5    




 

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