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

M. J. Manco-Johnson, R. Nuss, N. Key, C. Moertel, L. Jacobson, S. Meech, A. Weinberg, J. Lefkowitz

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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.

Original languageEnglish (US)
Pages (from-to)319-323
Number of pages5
JournalJournal of Pediatrics
Volume128
Issue number3
DOIs
StatePublished - Jan 1 1996

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Manco-Johnson, M. J., Nuss, R., Key, N., Moertel, C., Jacobson, L., Meech, S., Weinberg, A., & Lefkowitz, J. (1996). Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis. Journal of Pediatrics, 128(3), 319-323. https://doi.org/10.1016/S0022-3476(96)70274-3