en
Scientific article
Case report
English

Anti-protein S antibodies following a varicella infection: detection, characterization and influence on thrombin generation

Published inJournal of thrombosis and haemostasis, vol. 3, no. 6, p. 1243-1249
Publication date2005
Abstract

Postinfectious purpura fulminans is a rare disease. Varicella is one of the precipitating conditions and we recently observed such a case. The 4-year-old child was found to have a severe transient protein S deficiency. By enzyme-linked immunosorbent assay and surface plasmon resonance we first demonstrated that anti-protein S antibodies were present and also transient. Next we characterized the epitopes against which these antibodies were directed and found that they predominantly recognized the N-terminal part of protein S. Finally we showed by thrombography a transient dramatic hypercoagulable state as a result of thrombin being unregulated by the dynamic protein C inhibitory system: in vitro thrombin generation, in response to a low concentration of tissue factor, was almost insensitive to activated protein C up to 25 nmol L(-1) on day 4 while it was normally sensitive on day 42. For the first time, we demonstrated a temporal relationship between protein S deficiency, antibodies to protein S and hypercoagulability, thus supporting the pathogenic role of these antibodies.

Keywords
  • Autoantibodies/blood
  • Chickenpox/blood/complications
  • Child, Preschool
  • Enzyme-Linked Immunosorbent Assay
  • Epitope Mapping
  • Humans
  • Male
  • Protein S/immunology
  • Protein S Deficiency/etiology/virology
  • Purpura, Schoenlein-Henoch/etiology/virology
  • Surface Plasmon Resonance
  • Thrombin/biosynthesis
  • Thrombophilia/etiology/virology
  • Time Factors
Citation (ISO format)
REGNAULT, V et al. Anti-protein S antibodies following a varicella infection: detection, characterization and influence on thrombin generation. In: Journal of thrombosis and haemostasis, 2005, vol. 3, n° 6, p. 1243–1249. doi: 10.1111/j.1538-7836.2005.01270.x
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ISSN of the journal1538-7836
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