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An inquiry into the relationship between ABO blood group and thrombotic thrombocytopenic purpura
J F Staropoli1, C P Stowell, H H Tuncer
1Department of Pathology, Massachusetts General Hospital, Boston, MA, USA.
Background And Objectives:
ABO blood group accounts for up to 40% of the variability in plasma von Willebrand factor (VWF) levels, which vary in the rank order AB > B > A > O > Bombay. This may be due in part to the influence of ABO-associated oligosaccharides on the proteolysis of VWF by the metalloprotease ADAMTS13, which is markedly deficient in thrombotic thrombocytopenic purpura (TTP). Using ABO blood group as a surrogate for baseline VWF levels as well as susceptibility to proteolysis by ADAMST13, we set out to determine whether ABO blood group influences the clinical course of TTP.
Methods:
We conducted a retrospective analysis of the clinical course of 76 patients with primary, sporadic TTP treated at two institutions over the past 10 years.
Results:
We found no significant differences between group O and non-O patients with respect to presenting platelet count and lactate dehydrogenase concentration, maximum serum creatinine concentration, and total number of therapeutic plasma exchanges per episode.
Conclusions:
Substrate-related contributors to the highly variable phenotype and clinical course of TTP warrant further investigation.
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