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Blood, 15 September 2005, Vol. 106, No. 6, pp. 1988-1991.
Prepublished online as a Blood First Edition Paper on May 10, 2005; DOI 10.1182/blood-2005-02-0792.
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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Brief report
Bombay phenotype is associated with reduced plasma-VWF levels and an increased susceptibility to ADAMTS13 proteolysis
James S. O'Donnell,
Thomas A. J. McKinnon,
James T. B. Crawley,
David A. Lane, and
Michael A. Laffan
From the Imperial College London, Hammersmith Hospital Campus, Haematology Department, London, United Kingdom.
ABO blood group is an important determinant of plasma von Willebrand factor antigen (VWF:Ag) levels, with lower levels in group O. Previous reports have suggested that ABO(H) sugars affect the susceptibility of VWF to ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type-1 repeats-13) cleavage. To further test this hypothesis, we collected plasma from individuals with the rare Bombay blood group. VWF:Ag levels were significantly lower in Bombay patients (median, 0.69 IU/mL) than in groups AB, A, or B (P < .05) and lower than in group O individuals (median, 0.82 IU/mL). Susceptibility of purified VWF fractions to recombinant ADAMTS13 cleavage, assessed using VWF collagen-binding assay (VWF:CB), was increased in Bombays compared with either group O or AB. Increasing urea concentration (0.5 to 2 M) increased the cleavage rate for each blood group but eliminated the differences between groups. We conclude that reduction in the number of terminal sugars on N-linked glycan increases susceptibility of globular VWF to ADAMTS13 proteolysis and is associated with reduced plasma VWF:Ag and VWF:CB levels.
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