Priapism in homozygous sickle cell patients: important clinical and laboratory associations.

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.

Evaluation of clinical and laboratory correlates of sickle leg ulcers.

The exact mechanism for the occurrence of sickle leg ulcers (SLUs) has not been fully explained, although, popular opinion supports a multifactorial etio-pathogenetic process. Leg ulceration in sickle cell is a chronic and debilitating condition which is difficult to treat and may worsen the psychosocial impact of this illness. This study aims to evaluate the laboratory and clinical correlates of SLUs. One hundred sixty-seven patients who had been diagnosed with sickle cell anemia (homozygous S) had their steady-state hemoglobin concentration (Hb), hematocrit, white cell count, platelet count, serum bilirubin, and aspartate transaminase (AST) as well as frequency of crisis per annum evaluated with respect to their relationship to the occurrence of leg ulcers. They were aged 6-53 years (mean age 24.3 years), and prevalence of leg ulcer was found to be 2.75 per 1000 (2.54 per 1000 in females and 2.83 per 1000 in males). The independent sample t-test showed a significant difference in the serum AST levels in those with SLU (p = 0.029), though a positive correlation did not exist. Other predictors of disease severity found to have positive relationship with each other were the AST and total serum bilirubin 0.207 (p = 0.012); Hb and age 0.130 (p = 0.035); Hb and white cell count -0.159 (p = 0.010), white cell count and age -0.113 (p = 0.018). SLUs do not occur in patients with severe disease in sickle cell. The clinical and laboratory indicators of severe sickle cell disease do not correlate positively with the occurrence of SLU. Serum AST may have a relationship with leg ulceration in these patients. Environmental factors most likely play a major part in the etiopathogenesis of leg ulcer and this may require further studies in different sociocultural settings.