Use of hydroxyurea from childhood to adult age in sickle cell disease: semen analysis

Lukusa, Aimé Kazadi; Vermylen, Christiane

doi:10.3324/haematol.13659

Cited by 20 publications

(14 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Some investigators suggest that the length of HU therapy may correlate with the degree of sperm abnormalities. 82,83 In one study, of the patients who started HU in childhood, those who had received HU for 12 years or more had azoospermia. 83 However, most of these studies provide limited data because they involved small, retrospective populations.…”

Section: -78mentioning

confidence: 99%

See 1 more Smart Citation

Reproductive issues in sickle cell disease

Smith‐Whitley

2014

Blood

View full text Add to dashboard Cite

As medical advances improve survival, reduce disease-related morbidity, and improve quality of life, reproductive issues will take higher priority in the sickle cell disease (SCD) community. A wide variety of topics are addressed in this chapter, including fertility, gonadal failure, erectile dysfunction, and menstrual issues in SCD. Etiologies of impaired male fertility are multifactorial and include hypogonadism, erectile dysfunction, sperm abnormalities, and complications of medical therapies. Much less is known about the prevalence and etiology of infertility in women with SCD. Other reproductive issues in women included in this review are pain and the menstrual cycle, contraception, and preconception counseling. Finally, long-term therapies for SCD and their impact on fertility are presented.Transfusional iron overload and gonadal failure are addressed, followed by options for fertility preservation after stem cell transplantation. Focus is placed on hydroxyurea therapy given its benefits and increasing use in SCD. The impact of this agent on spermatogenesis, azoospermia, and the developing fetus is discussed. (Blood. 2014;124(24):3538-3543) IntroductionReproductive issues in women and men with sickle cell disease (SCD) include a wide array of complications that are relatively common; however, data from well-designed, large clinical studies are limited. Many studies are quite old, but remain relevant because they describe clinical complications and problems that persist in the SCD population today despite advances in medical therapy. Not unexpectedly, some of the reproductive issues in SCD arise due to chronic medical therapies that are used increasingly to prevent or manage SCD-related morbidity. Fertility in menInfertility in men with SCD has been studied more frequently than infertility in women and appears to have multiple causes, including hypogonadism, sperm abnormalities, and erectile dysfunction (ED) due to priapism. Although a delay in sexual maturation of 1.5-2 years, on average, occurs in adolescents and young adults with SCD, 1,2 most go on to have normal sexual maturation. 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.3 Multiple theories as to why these abnormalities develop in ma...

show abstract

Section: -78mentioning

confidence: 99%

“…14,[81][82][83][84] Whether these abnormalities are directly related to HU is unclear. Some investigators suggest that the length of HU therapy may correlate with the degree of sperm abnormalities.…”

Section: -78mentioning

confidence: 99%

Reproductive issues in sickle cell disease

Smith‐Whitley

2014

Blood

View full text Add to dashboard Cite

show abstract

“…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? 60 We believe that currently we do not have sufficient evidence to answer the above question when considering HLA-identical sibling HSCT for SCD.…”

Section: Is There Clinical Equipoise?mentioning

confidence: 99%

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

Nickel

Hendrickson

Haight

2014

Blood

View full text Add to dashboard Cite

Hematopoietic stem cell transplant (HSCT) is the only cure for sickle cell disease (SCD). HSCT using an HLA-identical sibling donor is currently an acceptable treatment option for children with severe SCD, with expected HSCT survival >95% and event-free survival >85%. HSCT for children with less severe SCD (children who have not yet suffered overt disease complications or only had mild problems) is controversial. It is important to consider the ethical issues of a proposed study comparing HLA-identical sibling HSCT to best supportive care for children with less severe SCD. In evaluating the principles of nonmaleficence, respect for individual autonomy, and justice, we conclude that a study of HLA-identical sibling HSCT for all children with SCD, particularly hemoglobin SS and Sβ0-thalassemia disease, is ethically sound. Future work should explore the implementation of a large trial to help determine whether HSCT is a beneficial treatment of children with less severe SCD.

show abstract

“…Whether these abnormalities are related to HU is not clear, but researchers opine that the length of HU therapy might correlate with the degree of sperm abnormalities [34,35].…”

Section: Hydroxyurea (Hu) and Fertility In Men With Scdmentioning

confidence: 99%

“…Sperm abnormalities, including azoospermia, oligozoospermia, increased morphologic abnormalities, and decreased motility, occur in men afflicted with SCD and receiving HU [20,[34][35][36]. Whether these abnormalities are related to HU is not clear, but researchers opine that the length of HU therapy might correlate with the degree of sperm abnormalities [34,35].…”

Section: Hydroxyurea (Hu) and Fertility In Men With Scdmentioning

confidence: 99%

Infertility Issues in Men with Sickle Cell Disease

AlDallal

2017

IPCB

View full text Add to dashboard Cite

Patients with sickle cell disease (SCD), especially males, face a key problem in the form of infertility. Apart from low serum testosterone, abnormalities involving accessory sex organs are also observed in these patients. Infertility is a known complication among males with SCD, and it comes about due to reasons such as impotence, relative primary gonadal failure, priapism, and delayed or impaired sexual development. Infertility manifests due to multiple causes, including primary gonadal failure (hypogonadism), erectile dysfunction (ED) as a result of priapism, sperm abnormalities, and delayed or impaired sexual development. The ejaculate volume, sperm density, sperm motility, and normal sperm morphology were significantly reduced in these patients as compared with normal patients. Most importantly, primary testicular failure is marked by low levels of testosterone and infertility results mainly from diseases or conditions that affect and destroy the testis. The low levels of testosterone lead to fertility reduction, which is aggravated by impotence, secondary to earlier priapism. Studies among mice have shown that increased doses of HU increase testicular germ cell apoptosis, decrease sperm count, induce testicular atrophy, increase abnormal sperm morphology, and decrease sperm motility. A multifactorial etiology is responsible for impaired male fertility and it includes sperm abnormalities, ED, hypogonadism, and effect of therapy on sperm function. This paper summarizes some clinical manifestations which play an important role in the development of infertility among men with SCD. The author suggests that prospective studies among males with SCD should be carried out using various end points to determine cellular and functional impairment in fertility.

show abstract

Use of hydroxyurea from childhood to adult age in sickle cell disease: semen analysis

Cited by 20 publications

References 5 publications

Reproductive issues in sickle cell disease

Reproductive issues in sickle cell disease

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

Infertility Issues in Men with Sickle Cell Disease

Contact Info

Product

Resources

About