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Evaluation of von Willebrand factor and ADAMTS-13 antigen and activity levels in sickle cell disease patients in Kuwait

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Abstract

Sickle cell disease (SCD) is a severe form of hemolytic anemia characterized by chronic hemolysis and is associated with increased thrombotic risk. Elevated von Willebrand factor (vWF) levels in SCD have been attributed to increased secretion and impaired processing by its cleaving protease ADAMTS-13. In this study we measured vWF and ADAMTS-13 antigen and activity levels in our SCD patients. Hematological and biochemical parameters for 59 SCD patients (20 children and 39 adults) were analyzed and compared to 59 age- and sex-matched controls. Commercially available ELISA kits were used to measure vWF and ADAMTS-13 antigen and activity levels in patients and controls. Patients had significantly higher levels of vWF (p < 0.006) and ADAMTS-13 activity (p < 0.006) compared to controls. When patients were analyzed according to age and genotype, adult patients (23 SS and 16 Sβ0thal) maintained higher vWF antigen levels (p < 0.001), but with reduced ADAMTS-13 activity to vWF:Ag ratio (p < 0.003) compared to controls. Pediatric patients (8 SS and 12 Sβ0thal) had comparable vWF antigen levels to controls (p > 0.05), but had higher levels of ADAMTS-13 activity (p < 0.011) and ADAMTS-13 activity to vWF:Ag ratio (p < 0.038). Age is an important factor to consider when vWF and ADAMTS-13 proteins are analyzed among our patients. Increased vWF in adult patients may be attributed to increased production and resistance of vWF to proteolysis rather than ADAMTS-13 deficiency. This outcome was not seen in pediatric patients as higher ADAMTS-13 activity maintained vWF antigen at comparable levels to normal controls.

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Acknowledgments

This work was supported and funded by Kuwait University Research Grant No. NM02/13. Special thanks to Mrs. Liena S. Abdulaziz for performing ELISA work. The authors also thank Rasha Abdullah, Hadeel Al-Muzaini and Nada Mustafa for their help in sample and data collection. The technical assistance of Mohammad Ezzat, Matra Salem and Mays Abdulhadi is appreciated.

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Authors and Affiliations

  1. Department of Medical Laboratory Sciences, Faculty of Allied Health Sciences, Kuwait University, P.O. Box 31470, 90805, Sulaibikhat, Kuwait

    Anwar Al-Awadhi

  2. Department of Pediatrics, Faculty of Medicine, Kuwait University, Sulaibikhat, Kuwait

    Adekunle Adekile

  3. Department of Pathology, Faculty of Medicine, Kuwait University, Sulaibikhat, Kuwait

    Rajaa Marouf

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  1. Anwar Al-Awadhi
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  2. Adekunle Adekile
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  3. Rajaa Marouf
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Correspondence to Anwar Al-Awadhi.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Al-Awadhi, A., Adekile, A. & Marouf, R. Evaluation of von Willebrand factor and ADAMTS-13 antigen and activity levels in sickle cell disease patients in Kuwait. J Thromb Thrombolysis 43, 117–123 (2017). https://doi.org/10.1007/s11239-016-1418-4

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  • DOI: https://doi.org/10.1007/s11239-016-1418-4

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