Hypogonadism in Patients with Sickle Cell Disease: Central or Peripheral?

Abstract

There is conflicting evidence in the literature on the etiology of hypogonadism in patients with sickle cell disease (SCD). A cross-sectional study was done to determine whether hypogonadism in male patients with SCD is due to primary testicular failure or secondary pituitary/hypothalamic dysfunction and assess the association between hypogonadism and serum ferritin levels. Hormonal assessment for serum concentrations of testosterone, follicle stimulating hormone (FSH) and luteinizing hormone (LH) was done for 34 men with SCD and their charts were reviewed for relevant clinical variables. Eight men (24%) were classified hypogonadal based on their serum testosterone levels. These men have significantly lower LH (p = 0.001) and FSH (p = 0.01) levels than normogonadal men, indicating a central etiology. There was no significant difference between hypogonadal and normogonadal men with respect to ferritin levels (p = 0.71). Our study indicates a central etiology of hypogonadism in patients with SCD. In this small study ferritin level was not significantly related to hypogonadism.

Growth and puberty in a prospective cohort of patients with sickle-cell anaemia: an assessment over ten years abstract

“…As adults, men with sickle-cell anaemia had significantly higher mean LH and FSH levels than controls and similar testosterone levels. These results contrast with the literature, since studies have shown low testosterone in adult men with sickle-cell anaemia, with gonadotropin levels that may reflect both hypogonadotropic hypogonadism and as hypergonadotropic 2,23 . A previous study found significantly lower testosterone averages in the sickle-cell anaemia group than in controls, but with similar averages of LH and FSH24.…”

Reproductive issues in sickle cell disease

“…However, up to 24% of men with SCD may develop hypogonadism, a clinical syndrome associated with poor testosterone production, infertility, ED, and poor libido. 3 Clinical characteristics include sparse facial, pubic, and axillary hair and small testicular size. Clinical laboratory findings are low testosterone levels with variable follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels.…”

“…4 Possible underlying pathophysiologic mechanisms of hypogonadism include disruptions in the hypothalamic-pituitary-gonadal axis leading to primary testicular failure. However, studies are inconsistent as to whether primary testicular failure 5,6 or secondary hypothalamic-pituitary dysfunction 3,4,[7][8][9] is the cause. A recent report found low serum testosterone levels in 8 of 34 men with SCD and all 8 had low FSH and LH levels, suggesting a central mechanism.…”

Infertility Issues in Men with Sickle Cell Disease

“…Hypogonadism, a condition linked with infertility, poor testosterone production, poor libido and erectile dysfunction, develops in up to 24% of men with SCD [14]. Patients show sparse facial, axillary and pubic hair and small testicular size.…”

“…The possible pathophysiologic mechanisms of hypogonadism are disruption in the hypothalamic-pituitary-gonadal axis, which leads to primary testicular failure [16,17], or secondary hypothalamic-pituitary dysfunction [14,15,18,19].…”