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Table of Contents   
CASE REPORT  
Year : 2013  |  Volume : 6  |  Issue : 5  |  Page : 583-585
Priapism: A chronic myeloid leukemia harbinger in exigency


1 Department of Medicine, PGIMER, New Delhi, India
2 Department of Medicine, Lady Hardinge Medical College and Associated Smt. Sucheta Kriplani Hospital, Dr. Ram Manohar Lohia Hospital, New Delhi, India

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Date of Web Publication3-Jun-2014
 

   Abstract 

Chronic myeloid leukemia may commonly present with atypical manifestations such as hearing loss or chloromas where the underlying diagnosis is revealed only on subsequent blood investigations. This case presented in the medicine emergency with one of the unusual complications of CML and so the diagnosis of CML was not suspected on the first instance. Prompt and comprehensive management of the patient not only saved him but also reiterated the fact that a rare presentation like priapism can sometimes forewarn of a more critical clinical condition.

Keywords: Chronic myeloid leukemia, hematological malignancy, priapism

How to cite this article:
Jana K, Aggarwal R, Gawande A, Lal M. Priapism: A chronic myeloid leukemia harbinger in exigency. Ann Trop Med Public Health 2013;6:583-5

How to cite this URL:
Jana K, Aggarwal R, Gawande A, Lal M. Priapism: A chronic myeloid leukemia harbinger in exigency. Ann Trop Med Public Health [serial online] 2013 [cited 2019 Nov 15];6:583-5. Available from: http://www.atmph.org/text.asp?2013/6/5/583/133752

   Introduction Top


Chronic myeloid leukemia (CML) is a common hematological malignancy in India with annual incidence of 0.8-2.2/100,000 population in males and 0.6-1.6/100,000 population in females. [1] Dragging sensation in left hypochondrium because of massive spleen generally brings the patient to the physician. Sometime, CML may present with an unexpected emergency like priapism when the diagnosis of CML is made retrospectively after blood investigations. The physicians must be aware of these uncommon manifestations of this common disorder for timely management.


   Case Report Top


The case we present here is about a 20-year-old male, nonsmoker presented to the medical emergency with sudden onset painful penile erection which had persisted for the last 4 h [Figure 1]. He denied any recent intercourse, trauma, use of illicit drugs, use of medications, and radiation therapy. There was no history of anorexia, weight loss, fever, sweating, chills, or bleeding from any site. The vital signs revealed a body temperature of 37.5°C, pulse 104 beats/min, blood pressure 130/74 mmHg and respiration rate of 20/min. He was conscious and oriented. The penis was erect, firm, and tender with superficial venous engorgement. The systemic examination revealed that the spleen was 8 cm below the left costal margin. Cardiovascular system and respiratory system were unremarkable. An initial working diagnosis of low-flow type priapism was made with splenomegaly. Investigations revealed hemoglobin 10.9 g/dl, white blood count 158,000/ml with polymorph 50%, myelocyte-35%, metamyelocyte- 5%, and myeloblast-2%, respectively. Peripheral smear showed immature leukocytes in various stages of differentiation [Figure 2] and erythrocyte sedimentation rate was 72 mm in 1 st h. Other investigations including liver functions, kidney functions, chest X-ray, and electrocardiography were unremarkable. Urgent intervention with cavernosa aspiration and epinephrine irrigation was done which relieved the pain and reduced the size of the penis considerably. Leukocytosis along with enlarged spleen prompted us to investigate this patient for CML. The BCR-ABL study came positive and leukocyte alkaline phosphatase score was decreased which confirmed our suspicion of CML as underlying etiology of priapism. Tablet imatinib 400 mg once daily along with tablet allopurinol 100 mg thrice daily and intravenous fluids to maintain hydration were started. Patient responded to the treatment and presently the leukemia is in remission. He is able to achieve an erection with manual stimulation and maintains the ability to ejaculate.
Figure 1: Priapism: Erect and painful penis

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Figure 2: Peripheral smear shows cells in different stages in granulocytopoietic development

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   Discussion Top


The term "priapism" is derived from the name of a Greek God Priapus whose symbol was an erect phallus and was considered the God of fertility and bountiful harvest. [2],[3] However, unlike the God of fertility this medical condition is associated with impotence in more than 50% cases. [4] Priapism is an acute medical emergency limited exclusively to men, characterized by prolonged, painful, and persistent abnormal erection of the penis unassociated with any sexual arousal. [5] Penis is innervated with both sympathetic and parasympathetic nervous system through cavernous nerves to affect the neurovascular events during erection and detumescence. The somatic nerves are primarily responsible for sensation and the contraction of the bulbocavernosus and ischiocavernosus muscles. [6] During erection neurotransmitters are released from the cavernous nerve terminals, which results in relaxation of smooth muscles of corpora cavernosa and dilatation of the arterioles and arteries causing increased blood flow. Detumescence is achieved by contraction of arteriolar and cavernosal smooth muscle, decreased blood pressure within cavernosal sinusoids, and increased venous outflow through emissary venules. [7] Priapism has incidence of 1.5/100,000 affecting any age group and particularly having a bimodal peak of incidence, between 5 and 10 years in children and 20-50 years in adults. [8]

Pathophysiology

Based on the underlying pathophysiological mechanism priapism can be classified into high- and low-flow types (ischemic priapism/veno-occlusive type). Low-flow type is the most common form where patient presents with a painful and rigid erection. The venous drainage from emissary venules is blocked which leads to intracavernosal stasis and accumulation of de-oxygenated blood within the cavernous tissue. This results in trabecular interstitial edema, ultrastructural changes in trabecular smooth muscle cells, and functional transformation of these cells to fibroblast-like cells. Irreversible erectile dysfunction may occur if the condition persists beyond 48 h or more. [9],[10] Low-flow priapism may be due to drug-induced, sickle cell disease and other hemoglobinopathies, thrombophilia states (protein C and other thrombophilias, lupus), hyperviscosity states (hyperleukocytosis, polycythemia), idiopathic or central nervous system (CNS)-mediated. [5],[9],[11],[12] High-flow or nonischemic/arterial priapism presents as painless erection following trauma to the perineum or the genitalia resulting in increased flow through the arteries. The veno-occlusive mechanism is usually intact and there is less chance of future erectile dysfunction. [13],[14] CML a common hematological malignancy sometimes surprises the physician in an emergency when it presents with uncommon presentations like priapism rather than its usual features. The diagnoses become apparent when hemogram suggests leukocytosis. Different mechanisms have been postulated for understanding the pathophysiology of priapism in CML. They are:

  1. Venous congestion of the corpora cavernosa resulting from mechanical pressure on the abdominal veins by the splenomegaly
  2. Sludging of leukemic cells in the corpora cavernosa and the dorsal veins of penis
  3. Infiltration of the sacral nerves with leukemic cells
  4. Infiltration of the CNS. [15],[16]


Recommendations for treatment of low-flow priapism include local penile or systemic anesthesia in the form of dorsal nerve block, circumferential penile block or subcutaneous local penile shaft block and intracavernosal instillation of a sympathomimetic agent like phenylephrine to induce detumescence. [17] Priapism that fails to respond to conservative measures is treated with surgical shunts designed to divert blood away from the corpus cavernosum.

This particular case had no prior symptoms suggestive of any hematological malignancy. The painful priapism brought this patient to the emergency where based on history and examination a diagnosis of ischemic type of priapism was made and hence decompression was timely done. The subsequent reports revealed leukocytosis, which probably caused venous occlusion and hence low-flow type of painful priapism.


   Conclusion Top


The key messages drawn from this case are that hematological malignancy can sometimes surprise emergency physicians with their atypical presentations like priapism. An early and prompt recognition of type of priapism helps not only in saving the vital sexual organ from long-term sequel, but also provides a substrate for finding the underlying etiology as CML in this case.

 
   References Top

1.Subramanian PG. Cytogenetic study in CML. Indian J Med Res 2012;135:12-3.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.Papadopoulos I, Kelâmi A. Priapus and priapism. From mythology to medicine. Urology 1988;32:385-6.  Back to cited text no. 2
    
3.Pryor J, Akkus E, Alter G, Jordan G, Lebret T, Levine L, et al. Priapism. J Sex Med 2004;1:116-20.  Back to cited text no. 3
    
4.Lue TF, Hellstrom WJ, McAninch JW, Tanagho EA. Priapism: A refined approach to diagnosis and treatment. J Urol 1986;136:104-8.  Back to cited text no. 4
[PUBMED]    
5.Berger R, Billups K, Brock G, Broderick GA, Dhabuwala CB, Goldstein I, et al. Report of the American Foundation for Urologic Disease (AFUD) Thought Leader Panel for evaluation and treatment of priapism. Int J Impot Res 2001;13 Suppl 5:S39-43.  Back to cited text no. 5
    
6.Bosch RJ, Benard F, Aboseif SR, Stief CG, Lue TF, Tanagho EA. Penile detumescence: Characterization of three phases. J Urol 1991;146:867-71.  Back to cited text no. 6
    
7.Dean RC, Lue TF. Physiology of penile erection and pathophysiology of erectile dysfunction. Urol Clin North Am 2005;32:379-95.  Back to cited text no. 7
    
8.Eland IA, van der Lei J, Stricker BH, Sturkenboom MJ. Incidence of priapism in the general population. Urology 2001;57:970-2.  Back to cited text no. 8
    
9.Keoghane SR, Sullivan ME, Miller MA. The aetiology, pathogenesis and management of priapism. BJU Int 2002;90:149-54.  Back to cited text no. 9
    
10.Spycher MA, Hauri D. The ultrastructure of the erectile tissue in priapism. J Urol 1986;135:142-7.  Back to cited text no. 10
[PUBMED]    
11.Fowler JE Jr, Koshy M, Strub M, Chinn SK. Priapism associated with the sickle cell hemoglobinopathies: Prevalence, natural history and sequelae. J Urol 1991;145:65-8.  Back to cited text no. 11
    
12.Hamre MR, Harmon EP, Kirkpatrick DV, Stern MJ, Humbert JR. Priapism as a complication of sickle cell disease. J Urol 1991;145:1-5.  Back to cited text no. 12
    
13.Bastuba MD, Saenz de Tejada I, Dinlenc CZ, Sarazen A, Krane RJ, Goldstein I. Arterial priapism: Diagnosis, treatment and long-term followup. J Urol 1994;151:1231-7.  Back to cited text no. 13
    
14.Cherian J, Rao AR, Thwaini A, Kapasi F, Shergill IS, Samman R. Medical and surgical management of priapism. Postgrad Med J 2006;82:89-94.  Back to cited text no. 14
    
15.Savona M, Talpaz M. Chronic myeloid leukemia: Changing the treatment paradigms. Oncology (Williston Park) 2006;20:707-11.  Back to cited text no. 15
    
16.Ponniah A, Brown CT, Taylor P. Priapism secondary to leukemia: Effective management with prompt leukapheresis. Int J Urol 2004;11:809-10.  Back to cited text no. 16
    
17.Rosenstein D, McAninch JW. Urologic emergencies. Med Clin North Am 2004;88:495-518.  Back to cited text no. 17
    

Top
Correspondence Address:
Ramesh Aggarwal
Department of Medicine, Lady Hardinge Medical College and Associated Smt. Sucheta Kriplani Hospital and Dr. Ram Manohar Lohia Hospital, New Delhi,
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1755-6783.133752

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