Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis

J Pediatr. 1996 Mar;128(3):319-23. doi: 10.1016/s0022-3476(96)70274-3.

Abstract

Objective: The objective of this study was to determine the cause of purpura fulminans, disseminated intravascular coagulation, or thrombosis in seven children with varicella. All children were found to have a lupus anticoagulant and acquired protein S deficiency. Thrombosis in five children was associated with presumed or documented infection with streptococcus.

Study design: Coagulation tests included determinations of the activated partial thromboplastin time, the prothrombin time, the dilute Russell viper venom time, the prothrombin F 1 + 2 fragment, the C4b-binding protein (C4b), total and free protein S antigen, and clotting activities of factors II, V, VII, and X and of protein C and protein S. Autoantibodies to phospholipids, cardiolipin, and protein S were determined in enzyme-linked immunosorbent assays.

Results: All children had a lupus anticoagulant and acquired protein S deficiency. Thrombosis in five children was associated with presumed or documented infection with streptococcus. All children transiently expressed free protein S deficiency, elevated levels of IgG, IgM, or both binding to protein S, the lupus anticoagulant, and increased concentration of the F 1+2 fragment. Four children also had antiphospholipid or anticardiolipin antibodies. In one child a purified IgG fraction cross-reacted with both protein S and a specific varicella antigen.

Conclusions: A subset of children with varicella infection, some of whom are coinfected with streptococcus, are prone to development of a lupus anticoagulant and an autoantibody to protein S, which results in acquired free protein S deficiency. Such children are at risk of having life-threatening thrombotic events.

Publication types

  • Comparative Study
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Antibodies, Antiphospholipid / analysis
  • Blood Coagulation Tests
  • Case-Control Studies
  • Chickenpox / complications*
  • Child
  • Child, Preschool
  • Disseminated Intravascular Coagulation / etiology
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Humans
  • Infant
  • Lupus Coagulation Inhibitor / analysis*
  • Male
  • Protein S Deficiency / etiology*
  • Purpura, Schoenlein-Henoch / etiology*
  • Risk Factors
  • Streptococcal Infections / complications
  • Streptococcus pyogenes
  • Thrombosis / etiology*

Substances

  • Antibodies, Antiphospholipid
  • Lupus Coagulation Inhibitor