Abstract
Background
Patients with sickle cell disease, frequently suffer from intense painful episodes. Till recently hydroxyurea was the only available medical therapy that approved for reduction of painful episodes.
Aims
To summarize the available data from randomized controlled trials that aim to evaluate the efficacy of newly approved L-glutamine (alters redox state of red blood cells [RBCs]) and crizanlizumab ((anti-P-selectin)) on vasoocclusive episodes in Sickle cell disease patients.
Methods
PubMed, Google Scholar, and EBSCO databases were systematically search for relevant articles. The terms Lglutamine, sickle cell disease, sickle cell anaemia, crizanlizumab and vaso-occlusive episodes were used.
Results
Out of Four-hundred seventy-two records, only three fulfilled the inclusion criteria. Two trials were aimed to evaluate the efficacy of L-glutamine therapy on the frequency of painful crises in sickle cell anaemia patients. Both studies showed that L-glutamine therapy significantly reduce the frequency of VOEs. Only one trial examined the ability of crizanlizumab on VOEs reduction, and showed crizanlizumab successful reduce the occurrence of VOEs.
Conclusion
Newer agent with different mechanism of action, such as Lglutamine, and crizanlizumab may consider if hydroxyurea not effective or not tolerable.