Article
Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura.
Blood - 12 Jul 2012
Lotta Luca A, Wu Haifeng M, Mackie Ian J, Noris Marina, Veyradier Agnes, Scully Marie A, Remuzzi Giuseppe, Coppo Paul, Liesner Ri, Donadelli Roberta, Loirat Chantal, Gibbs Richard A, Horne April, Yang Shangbin, Garagiola Isabella, Musallam Khaled M, Peyvandi Flora
Abstract excerpt
The quantification of residual plasmatic ADAMTS13 activity in congenital thrombotic thrombocytopenic purpura (TTP) patients is constrained by limitations in sensitivity and reproducibility of commonly used assays at low levels of ADAMTS13 activity, blunting efforts to establish genotype-phenotype correlations. In the present study, the residual plasmatic activity of ADAMTS13 was measured centrally by...
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