Manco-Johnson M J, Nuss R, Key N, Moertel C, Jacobson L, Meech S, Weinberg A, Lefkowitz J
Department of Pediatrics, University of Colorado Health Sciences Center, Denver 80262, USA.
J Pediatr. 1996 Mar;128(3):319-23. doi: 10.1016/s0022-3476(96)70274-3.
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.
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.
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.
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.
本研究旨在确定7名患水痘儿童发生暴发性紫癜、弥散性血管内凝血或血栓形成的原因。所有患儿均被发现存在狼疮抗凝物及获得性蛋白S缺乏。5名患儿的血栓形成与推测或确诊的链球菌感染有关。
凝血试验包括活化部分凝血活酶时间、凝血酶原时间、稀释蝰蛇毒时间、凝血酶原F1 + 2片段、C4b结合蛋白（C4b）、总蛋白S抗原和游离蛋白S抗原的测定，以及因子II、V、VII和X、蛋白C和蛋白S的凝血活性测定。采用酶联免疫吸附试验检测抗磷脂、抗心磷脂和抗蛋白S自身抗体。
所有患儿均有狼疮抗凝物及获得性蛋白S缺乏。5名患儿的血栓形成与推测或确诊的链球菌感染有关。所有患儿均短暂出现游离蛋白S缺乏、与蛋白S结合的IgG、IgM水平升高或两者均升高、狼疮抗凝物以及F1 + 2片段浓度增加。4名患儿还存在抗磷脂或抗心磷脂抗体。1名患儿的纯化IgG组分与蛋白S和一种特定的水痘抗原发生交叉反应。
一部分水痘感染患儿，其中一些合并链球菌感染，易发生狼疮抗凝物及抗蛋白S自身抗体，导致获得性游离蛋白S缺乏。这些患儿有发生危及生命的血栓事件的风险。
