Priapism associated with asplenic state

Atala, Anthony; Amin, Mohammad; Harty, James I.; Liu, Yong K.; Keeling, Marie M.

doi:10.1016/0090-4295(92)90393-b

Cited by 10 publications

(4 citation statements)

References 16 publications

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“…As our data about adverse vascular events come only from families with hereditary spherocytosis, we searched Medline for reports of adverse vascular events occurring more than 1 month after splenectomy, regardless of the indication for surgery. We found 80 citations [1,7–85] documenting nine different, although not necessarily independent, adverse vascular events after splenectomy performed for more than 14 different indications. (A tabulation of these data is available via e‐mail request to RFS.…”

Section: Discussionmentioning

confidence: 99%

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Delayed adverse vascular events after splenectomy in hereditary spherocytosis

Schilling

Gangnon

Traver

2008

Journal of Thrombosis and Haemostasis

103

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To cite this article: Schilling RF, Gangnon RE, Traver MI. Delayed adverse vascular events after splenectomy in hereditary spherocytosis. J Thromb Haemost 2008; 6: 1289-95.Summary. Background: It is probable that the variety and frequency of delayed adverse vascular events after splenectomy are underappreciated. Splenectomy is performed for a wide variety of conditions, and delayed postsplenectomy hazards are not often studied. Objective: To estimate the relative risk of adverse vascular events in members of hereditary spherocytosis families who have or have not had a splenectomy. Methods: Members of families in which hereditary spherocytosis exists were systematically questioned about adverse vascular events. Results: The cumulative incidence of arterial and venous events at age 70 years was greater in persons who had undergone a splenectomy for spherocytosis (arterial, 22% females, 32% males; venous, 20% females, 19% males) than in affected persons who did not undergo splenectomy (arterial, 3% females, 2% males; venous, 6% females, 4% males) or non-affected family members (arterial, 10% females, 17% males; venous, 4% females, 12% males). Affected subjects who undergo splenectomy are at greatly increased risk of arterial events as compared to affected subjects who do not undergo splenectomy [arterial, hazard ratio (HR) 7.2, 95% confidence interval (CI) 2.8-17.2; venous, HR 3.3, 95% CI 1.1-9.8]. Conclusion: There is a significant, long-lasting, increased risk of adverse arterial and venous thromboembolic events after splenectomy performed for hereditary spherocytosis. A review of the literature indicates that this is also true when splenectomy is performed for several other indications.

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Section: Discussionmentioning

confidence: 99%

“…A review of 21 patients with priapism [69] found 10 patients with sickle cell disease, but three of the other 11 had had the spleen removed for trauma months or years prior to the onset of priapism. We found seven other reports of priapism after splenectomy performed for a variety of conditions [22–24,37,42,75,84].…”

Section: Discussionmentioning

confidence: 99%

Delayed adverse vascular events after splenectomy in hereditary spherocytosis

Schilling

Gangnon

Traver

2008

Journal of Thrombosis and Haemostasis

103

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“…Priapism has been reported in patients with a wide variety of hemolytic anemias beside SCD (Table 1), including thalassemia [22–27], hereditary spherocytosis, paroxysmal nocturnal hemoglobinuria (PNH), unstable hemoglobin disorders [31–33], glucose‐6 phosphate dehydrogenase deficiency [34,35], glucose‐6 phosphate isomerase deficiency [36], and congenital dyserythropoietic anemia [37]. In many of these cases, the onset of priapism followed splenectomy, leading to the suggestion that asplenia might be a risk factor for priapism [38,39]. Asplenia, a common feature of SCD, promotes thrombocytosis and leukocytosis.…”

Section: Clinical Complications Of Scdmentioning

confidence: 99%

Priapism in Sickle-Cell Disease: A Hematologist’s Perspective

Kato

2012

The Journal of Sexual Medicine

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Introduction Priapism is a familiar problem to hematologists, well known for its association with sickle cell disease. It also occurs in a variety of other hematological illnesses, nearly all forms of congenital hemolytic anemia, including other hemoglobinopathies and red blood cell membranopathies and enzymopathies. Aim Provide urologists with a comprehensive review of priapism in sickle cell disease, with an emphasis on the perspective of a practicing hematologist. Methods Medline searches through July 2010 were conducted using the terms priapism, erectile dysfunction, and sickle cell. Main Outcome Measure Expert opinion was based on review of the medical literature related to this subject matter. Results In men with sickle cell disease, large epidemiological studies have linked the risk of priapism to clinical markers of the severity of intravascular hemolysis. Extracellular hemoglobin and arginase released during hemolysis has been implicated in reducing nitric oxide bioavailability, although the relevance of hemolysis to vascular dysfunction has been challenged by some scientists. Consistent with the role of impairment of the nitric oxide axis, mice genetically deficient in nitric oxide production have also been shown to develop priapic activity. Provocative new data indicates that hemolysis-linked dysregulation of adenosine signaling in the penis contributes to priapism in sickle cell mice. Serious questions have arisen regarding the efficacy of mainstays of textbook dogma for treatment of acute severe priapism, including intravenous fluids, alkalinization and exchange transfusion, and there is increasing acceptance for early aspiration and irrigation of the corpus cavernosum. Conclusions For sickle cell patients with recurrent priapism, there is very limited evidence for a medical prophylaxis role for hydroxyurea, etilefrine, pseudoephedrine, leuprolide, sildenafil, and other agents. Recent publications have highlighted nitric oxide and adenosine signal transduction pathways as worthy of additional research. Research and clinical management of sickle cell priapism is strengthened by multidisciplinary collaboration between hematologists and urologists.

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“…15 Asplenia has been implicated as a precipitating factor for the priapism occurrence. 23,26,39,40 This reflects a loss of the role that spleen plays as part of the reticuloendothelial system.…”

Section: Discussionmentioning

confidence: 99%

Clinical and laboratory parameters, risk factors predisposing to the development of priapism in sickle cell patients

Alkindi

Almufargi

Pathare

2019

Exp Biol Med (Maywood)

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Although sickle cell disease is very common in Oman, priapism is a relatively rare complication of this disease. This study was aimed to identify the clinical and laboratory risk factors that predispose sickle cell disease patients to priapism. In a retrospective, case-control study, data on 21 male sickle cell disease patients, with priapism, were compared to 20 age and sex-matched sickle cell disease patients without priapism from the hospital medical records. Specifically, the demographical, hematological, biochemical, and clinical parameters including complications attributable to priapism were studied. Means for continuous measures and independent t-test were used to evaluate the association between the parameter studied and the occurrence of priapism. Priapism occurrence was associated with low basal hemoglobin (Hb), along with an increase in other parameters such as white blood cell count (WBC) ( P = 0.010), platelet count ( P = 0.001), reticulocyte count ( P < 0.001), mean corpuscular volume (MCV) ( P < 0.001), and mean corpuscular hemoglobin (MCH) ( P < 0.001). Biochemical laboratory parameters revealed an association with an increased total bilirubin ( P < 0.001). Patients with priapism were more likely to have acute chest syndrome, stroke, pulmonary hypertension, asplenia ( P = 0.006), gallbladder stones, and consequently cholecystectomy. Blood exchange and Penile aspiration were the most frequent medical therapies ( P = 0.010 and P < 0.001 respectively). Patients with sickle cell disease who presented with priapism were young adults with low Hb, high WBC’s, platelets, reticulocytes, MCV, MCH, bilirubin, and LDH. These findings suggest an association of priapism with increased hemolysis. Impact statement Sickle cell disease is prevalent throughout the world with a 5.8% incidence of sickle cell trait among the Omani population, yet priapism was a relatively rare complication. Penile erection and detumescence are complex physiological processes, which require a delicate neurohormonal and cardiovascular response. In this cohort, young SCD patients developed priapism with a low incidence of complications. These patients had high WBCs, platelets, bilirubin, LDH, and reticulocytes with low hemoglobin as compared to controls, suggesting an increased hemolytic process. Favorable outcome was dependent on early intervention, with almost half of these patients needing surgical intervention.

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Priapism associated with asplenic state

Cited by 10 publications

References 16 publications

Delayed adverse vascular events after splenectomy in hereditary spherocytosis

Delayed adverse vascular events after splenectomy in hereditary spherocytosis

Priapism in Sickle-Cell Disease: A Hematologist’s Perspective

Clinical and laboratory parameters, risk factors predisposing to the development of priapism in sickle cell patients

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