Advances in managing sickle cell anemia: a systematic review


  • Isaac Johnson Ajeh Drug Manufacturing Unit, Department of Pharmaceutical Technology and Raw Materials Development, National Institute for Pharmaceutical Research and Development (NIPRD), Abuja, Nigeria
  • Ozhe Sunday Isaac Pediatric Hematology/Oncology Unit, Department of Pediatrics, Dalhatu Araf Specialist Hospital, Laa, Nigeria



Acute chest syndrome, Gene therapy, Haemoglobin, Sickle cell anemia, Vaso-occlusion, Niprisan


Background: Despite the acceptance and approval of several medications and techniques to reduce vaso-occlusive episodes (VOEs), Hydroxyurea along with other analgesics have remained the primary treatment option for Sickle Cell Anaemia (SCA) in Nigeria. However, in terms of cost-effectiveness and fewer side effects, Niprisan® remains the preferred option. In this review, we discussed new drugs/technologies as well as previously approved medications that could ameliorate SCAaside from the options available in Nigeria; and we hope to inspire our readers by providing insights into new inventions to overcome current challenges in the field.

Methods: This review involved a comprehensive examination of existing literature on SCA treatments; specifically focusing on new pharmaceutical developments, innovative technologies, and previously approved medications on Google Scholar, PubMed, ResearchGate, EMBASE, and Cochrane database using SCA, novel therapies, Niprisan®, haematopoietic stem cell transplant, and gene therapy as search items. Additionally, the references of some retrieved articles were also searched. The literature retrieved included review articles, meta-analyses, clinical trials, and original research papers.

Conclusions: Advanced insights into the cellular and molecular basis of the sickle cell disease processes have unveiled several established/potential drug targets on which newer SCA therapies are based. These newer therapies have varied mechanisms ranging from Fetal haemoglobin (HbF) induction, RBC membrane stabilization, oxidative stress reduction, adhesion inhibition, reduction of inflammation, prevention of polymerization, and enhanced flow dynamics to gene-directed therapies with the potential for cure. This expounded review has highlighted real progress in SCA treatment. However, an improved survival rate will depend on the participation of clinical sites across the globe, as well as the availability of funds to support studies needed to confirm the safety and efficacy of these


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How to Cite

Ajeh, I. J., & Isaac, O. S. (2024). Advances in managing sickle cell anemia: a systematic review. The Nigerian Journal of Pharmacy, 58(1), 49–58.