Received January 10, 2024
Accepted July 25, 2024

With the improvement in survival and the reduction in morbidity related to sickle cell disease (SCD), aspects related to reproductive health are emerging as a priority in the care of affected people. We conducted a systematic review looking for evidence describing the functional ovarian reserve levels in women with sickle cell disease. To locate studies, a search was performed in electronic databases, in addition to preprint servers and reference lists of selected publications. Two independent reviewers searched, and the risk of bias in the selected studies was assessed using the Newcastle–Ottawa scale. 1,086 records were initially retrieved, and one article was identified after consulting the reference lists of the screened articles, only four articles met the eligibility criteria. The quality of evidence was rated very low due to the design of the studies; however, the risk of bias was considered low. These are recent studies published between 2015 and 2021, whose main methodology was a cross-sectional or case-control study. All studies reported lower anti-mullerian hormone levels in women with sickle cell disease. Although lower, anti-mullerian hormone levels were within the normal range in young women with sickle cell disease only with supportive care, in those who used hydroxyurea, a decrease in ovarian reserve was observed. support insufficient evidence support a causal relationship between sickle cell disease and reduced ovarian reserve. From anti-mullerian hormone values a trend towards lower levels in women with sickle cell disease compared to healthy women, as reported in the four studies evaluated. Studies that analyze the ovarian reserve based on imaging and biochemical parameters are an important future focus.