Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura / 中华医学杂志(英文版)
Chinese Medical Journal
;
(24): 818-822, 2004.
Article
in English
| WPRIM
| ID: wpr-284900
ABSTRACT
<p><b>BACKGROUND</b>Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. In this study we investigated the von Willebrand factor-cleaving protease (vWF-cp) activity deficiency in patients with TTP.</p><p><b>METHODS</b>The plasma or serum vWF-cp activity was measured using a sensitive enzyme-linked immunosorbent assay (ELISA) by detecting the residual collagen binding activity (R-CBA) of von Willebrand factor (vWF) before and after digestion by vWF-cp. Multimers of vWF in plasma of patients with TTP were also analyzed by SDS-agarose electrophoresis. Moreover, the serum vWF-cp activities were compared between the patients with TTP and those with tumors.</p><p><b>RESULTS</b>The coefficient of variation for intra-batch and inter-batch of the assay were 3.60% and 8.35%. The plasma and serum vWF-cp activity in healthy individuals were (78.79 +/- 9.17)% (n = 30) and (79.47 +/- 10.78)% (n = 53), respectively, while the plasma vWF-cp activity in 5 patients with TTP was markedly decreased [(21.83 +/- 19.98)%, P < 0.001]. The unusually large vWF multimers were observed in two plasma samples of the patients with TTP. Although the vWF-cp activities in patients with benign and malignant tumors were also decreased (P < 0.03 and P < 0.001, respectively), they were relatively high in comparison with that of TTP patients (P < 0.001).</p><p><b>CONCLUSION</b>Measurement of the vWF-cp activity using R-CBA is a simple and rapid method for diagnosing TTP. The vWF-cp activity in patients with TTP was markedly lower than those of patients with tumors.</p>
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Purpura, Thrombotic Thrombocytopenic
/
Blood
/
Metalloendopeptidases
/
ADAM Proteins
/
ADAMTS13 Protein
/
Neoplasms
Limits:
Adolescent
/
Adult
/
Aged
/
Female
/
Humans
/
Male
Language:
English
Journal:
Chinese Medical Journal
Year:
2004
Type:
Article
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