Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis☆,☆☆,★,★★
Section snippets
Clinical methods
Laboratory investigations were performed on blood samples from seven children referred to one of the authors for evaluation of purpura fulminans, disseminated intravascular coagulation, or thrombosis in association with varicella. An eighth child, with signs of recent varicella without thrombosis, was an identical twin of one of the study children. Control values were derived on blood samples from 22 healthy children who had no personal or family history of bleeding or thrombotic disorders and
RESULTS
Clinical information about the children is shown in the Table. On admission to the hospital, child 1 received 10 ml of fresh frozen plasma per kilogram of body weight; protein S was undetectable immediately before and after. Children 3 to 7 received therapeutic doses of heparin. Urokinase, 4400 units/kg per hour for 24 to 48 hours, was administered to children 4, 6, and 7, with simultaneous administration of heparin at 10 units/kg per hour. After completion of the urokinase dosing, the heparin
DISCUSSION
We previously reported one case of purpura fulminans in a child with varicella, acquired protein S deficiency, and a lupus anticoagulant.4 Acquired protein S deficiency, an IgG anticardiolipin antibody, and progressive thromboembolism have since been confirmed in children with varicella.5, 7
We have now studied six additional children with varicella-associated thrombosis, DIC, or purpura fulminans, as well as one symptom-free sibling, for a lupus anticoagulant, other antiphospholipid antibodies,
Acknowledgements
We acknowledge Dr. Taru Hays for patient referrals, Dr. William E. Hathaway for critically reviewing the manuscript, and Susan Clarke and the Special Coagulation Laboratory of University Hospital, Denver, Colo., for technical support.
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From the Departments of Pediatrics and Pathology, University of Colorado Health Sciences Center and Children's Hospital, Denver, and the University of Minnesota and Children's Medical Center, St. Paul
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Supported by the Pediatric Clinical Research Center (grant RR-00069).
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Reprint requests: Marilyn J. Manco-Johnson, MD, C-220, University of Colorado Health Sciences Center, 4200 East 9th Ave., Denver, CO 80262.
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