Role of L- glutamine and crizanlizumab in sickle cell anaemia painful crisis reduction

Laila Ahmed Ayoub, Abdulaziz Abdulrahman A Bedaiwi, Turki Saleh S Alanazi, Nawaf Saeed A. Almalki, Raghad Shiraz M. Alharthi, Rana Eidhah M. Almalki, Eilaf Khaled A. Alsirhani, Khalid Khalaf M. Alanazi, Rakan Mohammed A. Bedaiwi, Hanan Rizqallah A. Alharbi, Manal Mohammed E Alhawiti, Mohammed Abdullah M Albalawi, Jumanah Faisal I Algoufi, Waleed Khaled Alshehri, Abdulwahab Mousa R Albalawi, Rahaf Ahmed A Alharthy, Ibrahim Mahmoud Ajwah, Waseem Babur Rehmatullah

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 vaso-occlusive episodes in Sickle cell disease ‎ patients.

Methods
PubMed, ‎Google Scholar, and EBSCO ‎ databases were ‎‎systematically search for relevant articles. The terms ‎ ‎ ‎ L-glutamine, 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 ‎L-glutamine, ‎and crizanlizumab may consider if ‎hydroxyurea not effective or not ‎tolerable.
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