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

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.

• Authors: M J Manco-Johnson, R Nuss, N Key, C Moertel, L Jacobson, S Meech, A Weinberg, J Lefkowitz
• Journal: The Journal of pediatrics (J Pediatr) Vol. 128 Issue 3 Pg. 319-23 (Mar 1996) ISSN: 0022-3476 [Print] United States
• PMID: 8774497 (Publication Type: Comparative Study, Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Antibodies, Antiphospholipid
• Lupus Coagulation Inhibitor

Topics

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