Priapism as a Complication of Sickle Cell Disease

Hamre, Merlin; Harmon, Edwin; Kirkpatrick, Dahlia; Stern, Marta J.; Humbert, James R.

doi:10.1016/s0022-5347(17)38229-0

Cited by 90 publications

(41 citation statements)

References 60 publications

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“…There is a suggestion that successful surgical intervention is less likely to preserve potency than successful conservative intervention. In a comprehensive review by Hamre et al [9] the preservation of potency appeared to be more successful with conservative, i.e. non surgical treatment; 48 of 51 patients (94%) treated conservatively maintained erectile potency, contrasting with only 25 of 43 (58%) retaining erectile potency after surgery.…”

Section: Discussionmentioning

confidence: 99%

Priapism in sickle‐cell disease; incidence, risk factors and complications – an international multicentre study

et al. 2002

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Objective To define the incidence, risk factors and complications of priapism in a large population of patients with sickle‐cell anaemia in five centres in the UK and Nigeria, as priapism is common among these patients, but the precise characteristics of the condition in this population are poorly documented.Patients and methods A questionnaire was developed and administered to patients with sickle‐cell disease. Questions were designed to define the incidence, nature, precipitants, duration, treatment and complications of priapism. A distinction was made between acute (severe) priapism and the recurrent, ‘stuttering’ type.Results The questionnaire was completed by 130 patients (mean age 25 years, sd 11, range 4–66) from the five centres; 102 (78%) were homozygous Hb SS genotype, 19 (15%) were Hb SC genotype and two (1.5%) were Hb Sα−thalassaemia. Of the patients, 46 (35%) reported a history of priapism, and of these, 33 (72%) had a history of stuttering priapism, while 24 (52%) had had an acute episode of priapism. The mean age of onset of priapism was 15 years, with 75% of patients having the first episode before their 20th birthday. Sexual activity was the most frequent precipitating factor, with fever and/or dehydration being the next most common. Of the 46 patients, 10 (21%) with a history of priapism reported having erectile dysfunction. A similar proportion reported dissatisfaction with sexual intercourse, including a fear of engaging in sexual activity.Conclusion The incidence of priapism among patients with sickle‐cell anaemia is high (35%). The implications of priapism for erectile and sexual function are significant and documented in this large series. The treatment of this condition in these patients remains unstandardised. This study highlights the need for an increased awareness of the problems associated with priapism among patients, families and medical professionals.

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Section: Discussionmentioning

confidence: 99%

Priapism in sickle‐cell disease; incidence, risk factors and complications – an international multicentre study

et al. 2002

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“…The clinical manifestation of priapism appears commonly as stuttering and major attacks [5][6][7]12,13].…”

Section: Introductionmentioning

confidence: 99%

Follow‐up of sickle cell disease patients with priapism treated by hydroxyurea

Saad¹,

Lajolo²,

Gilli³

et al. 2004

American J Hematol

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Hydroxyurea is one of the most successfully used therapies for sickle cell disease. Results of many clinical trials point to hydroxyurea administration for patients with frequent painful crises and acute chest syndrome. Priapism is one of the complications that could be prevented by hydroxyurea, but there are few reports demonstrating the results. Since November 1993, hydroxyurea has been used in our clinic for preventing priapism in patients with stuttering or major attacks who are still capable of achieving intercourse on demand. Five patients were enrolled in the study, and 4 cases benefited by this treatment. After the initial treatment for the acute attack, all five patients developed stuttering priapism. Hydroxyurea was then introduced at the initial dose of 10 mg/kg, and as the hydroxyurea dosage increased, the number or length of priapism episodes decreased. One to two months after the maximal dose (20-35 mg/kg) was introduced, the episodes disappeared. In two patients, we were forced to administer over 30 mg hydroxyurea/kg to abort the episodes, and, in another patient, 25 mg/kg was necessary. All patients present normal sexual activity. Hydroxyurea was discontinued in two patients, but stuttering priapism reappeared. Hydroxyurea was then re-introduced, and priapism disappeared. One patient, using 20 mg hydroxyurea/kg, had a 6-year remission of priapism after hydroxyurea administration; however, he experienced stuttering priapism, 1 month before a major attack, that progressed to impotence. During that month, he did not seek medical attention. In conclusion, the data here presented suggests that hydroxyurea may prevent priapism attacks in sickle cell disease, probably at higher doses than usually prescribed for painful crisis prevention. Am.

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“…The rationale for androgen deprivation lies in the notion that stuttering priapism often begins with the erections of rapid eye movement (REM) sleep, 2,20 and that nocturnal penile tumescence (NPT; that is, spontaneous erections) are testosterone dependent. 21 In the sickle cell study by Emond et al, 3 53 of 54 patients initiated stuttering in association with sleep; and 61% of major priapic episodes were preceded by stuttering.…”

Section: Discussionmentioning

confidence: 99%

Priapism of ambiguous classification in a sickle cell patient

2000

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We report a case of recurrent priapism in a sickle cell patient who had undergone physiological transformation from a low-¯ow state to a mixed high-¯owalow-¯ow picture. An oral alphaadrenergic agonistahistamine-1 antagonist combination maintained a balance of potency without stutter in the latter state, whereas an oral alpha-adrenergic agonist in the early part of his course did not prevent priapic progression. Concomitant use of a non-steroidal anti-androgenic agent rendered him impotent despite good libido. Other etiologies should be considered in the setting of recurrent priapism and failed standard therapies; duplex ultrasound is useful in order to delineate the hemodynamics. This case supports contentions that priapism is a spectrum phenomenon, and raises questions regarding the mechanism by which alpha-adrenergic agents prevent priapic progression.

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Priapism as a Complication of Sickle Cell Disease

Cited by 90 publications

References 60 publications

Priapism in sickle‐cell disease; incidence, risk factors and complications – an international multicentre study

Priapism in sickle‐cell disease; incidence, risk factors and complications – an international multicentre study

Follow‐up of sickle cell disease patients with priapism treated by hydroxyurea

Priapism of ambiguous classification in a sickle cell patient

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