Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura.

@article{Lotta2012ResidualPA,
  title={Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura.},
  author={Luca Andrea Lotta and Haifeng M. Wu and Ian Mackie and Marina Noris and Agn{\`e}s Veyradier and Marie Scully and Giuseppe Remuzzi and Paul Coppo and Ri J Liesner and Roberta Donadelli and Chantal Loirat and Richard A. Gibbs and April Horne and Shangbin Yang and Isabella Garagiola and Khaled M. Musallam and Flora Peyvandi},
  journal={Blood},
  year={2012},
  volume={120 2},
  pages={440-8}
}
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 surface-enhanced laser desorption/ionization time-of-flight mass spectrometry (limit of… CONTINUE READING
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