Priapism in Homozygous Sickle Cell Patients: Important Clinical and Laboratory Associations

Abstract

Objective: To evaluate the relationship between the occurrence of priapism and important steady-state clinical and laboratory parameters in homozygous sickle cell disease (SCD). Subjects and Methods: Steady-state clinical and laboratory data were obtained from the medical records of 126 male patients seen in the clinic over a 7-year period. Estimated prevalence rates, correlation coefficients and independent t tests were calculated to assess the relationship between priapism and several important clinical and laboratory indices. Patient data on age, haemoglobin concentrations, the frequency of crises per annum, leucocyte counts, platelet counts, serum bilirubin and aspartate transaminase were evaluated. Results: The prevalence of priapism was determined to be 21.4%, and 22.2% of those affected had erectile dysfunction. There was a significant positive correlation between priapism and older age (p = 0.049) and lower leucocyte counts (p = 0.008). There was no significant relationship with other clinical or laboratory indices. Conclusion: About 1 in 4 of all homozygous older SCD patients had priapism, and an approximately similar ratio developed erectile dysfunction; they also had lower steady-state leucocyte counts. Other clinical and laboratory indicators of disease severity in SCD did not positively correlate with the occurrence of priapism, and this may imply an alternative pathogenetic mechanism.

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

“…In men with SCD, vaso-occlusion and recurrent testicular infarction may contribute to hypogonadism [122,123]. Erectile dysfunction has been reported in as many as 21% to 35% and is likely a sequela of recurrent episodes of priapism [124,125]. Osegbe et al demonstrated that adult males with SCD, not taking hydroxyurea, have lower sperm count, motility and The participants assigned to hydroxyurea treatment had lower annual rates of crises than the participants given placebo (median, 2.5 vs. 4.5 crises per year, P < 0.001).…”

Reproductive issues in sickle cell disease

“…[18][19][20] The etiology of ED is unclear, but is largely the result of prolonged or recurrent priapism. Management of ED due to priapism depends on the extent of penile tissue fibrosis.…”

The ethics of a proposed study of hematopoietic stem cell transplant for children with “less severe” sickle cell disease

“…39,51 Although the long-term prognosis of children with initially less severe SCD may be better (especially in the future with continued improvements in supportive care), an individual patient's disease course is likely to involve significant problems. The specific risk of infertility secondary to HSCT also has to be balanced with the risk of reproductive problems related to SCD complications (impotence from priapism for men, [52][53][54] increased pregnancy complications for women [55][56][57] ) and also weighed against hydroxyurea therapy toxicity. 44,58,59 Thus, given the serious problems caused by SCD, some have questioned: Is the medical community actually harming patients with SCD by not offering them a curative therapy (HSCT) early and forcing these patients to live with a disease that causes high morbidity and premature mortality?…”