Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis^{, , ,}

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• 作者：Marilyn J. Manco-Johnson ; Rachelle Nuss ; Nigel Key ; Christopher Moertel ; Linda Jacobson ; S ; ra Meech ; Adriana Weinberg ; Jerry Lefkowitz
• 刊名：The Journal of Pediatrics
• 出版年：1996
• 出版时间：March 1996
• 年：1996
• 卷：128
• 期：3
• 页码：319-323
• 全文大小：456 K

文摘

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. (J PEDIATR 1996;128:319-23)