Sickle cell disease (SCD) is one of the most common hereditary diseases in the world. It leads to hemolytic anemia and painful vaso-occlusive crises that can damage target organs at the cardiopulmonary, cerebrovascular, and renal levels. SCD has also significant consequences on reproductive functions and fertility. Moreover, the treatments designed to alleviate and reduce vaso-occlusive crises directly impact male reproductive functions. Nevertheless, literature assessing the impact of SCD and its treatments on male reproductive functions remains limited and lacks robust evidence. A systematic review of the literature following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendation was carried out on the reproductive functions of men with SCD and the reproductive options available to them. Most studies have found that men with SCD frequently exhibit impaired sperm parameters. In addition, hydroxyurea (HU), proposed to relieve and reduce vaso-occlusive crises, is also known to impact male reproductive functions, and the reversibility of these consequences on sperm parameters remains hypothetical. Hematopoietic stem cell transplantation (HSCT) is currently the only curative treatment. However, conditioning treatments are highly gonadotoxic and can permanently alter spermatogenesis. Young men with SCD should therefore be informed about fertility at an early stage, and fertility preservation should be discussed in pubescent men, especially if treatment with HU or HSCT is to be initiated. In prepubertal boys about to undergo HSCT, immature testicular tissue freezing should be discussed, even though this technique is still experimental.

Keywords: fertility preservation; hydroxyurea; male reproductive functions; sickle cell disease; sperm parameters