Autoimmune Protein S Deficiency and Deep Vein Thrombosis after Chickenpox

Peyvandi, Flora; Faioni, Elena M.; Moroni, Gian Alessandro; Rosti, Alberto; Leo, Luigi; Moia, Macro

doi:10.1055/s-0038-1650245

Cited by 11 publications

(11 citation statements)

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“…About 50 cases with purpura fulminans post‐varicella have been reported. Most cases were associated with a transient and severe PS deficiency as a result of anti‐PS antibodies [1–7]. These autoantibodies were found to be IgG and/or IgM [3,5].…”

Section: Discussionmentioning

confidence: 99%

“…Peyvandi et al . also observed PS deficiency, presumably autoimmune, in a patient with deep vein thrombosis after varicella [6]. Josephson et al .…”

Section: Introductionmentioning

confidence: 98%

“…first reported the occurrence of thrombosis with a transient deficiency of PS ascribed to the presence of a circulating autoantibody to PS [3]. Since then, a few reports have confirmed the presence of anti‐PS antibodies after varicella associated with severe thrombotic complications [4–7]. Indeed Levin et al .…”

Section: Introductionmentioning

confidence: 99%

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Anti‐protein S antibodies following a varicella infection: detection, characterization and influence on thrombin generation

Régnault

Boehlen²,

Özşahin

et al. 2005

Journal of Thrombosis and Haemostasis

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Summary. Postinfectious purpura fulminans is a rare disease. Varicella is one of the precipitating conditions and we recently observed such a case. The 4-year-old child was found to have a severe transient protein S deficiency. By enzyme-linked immunosorbent assay and surface plasmon resonance we first demonstrated that anti-protein S antibodies were present and also transient. Next we characterized the epitopes against which these antibodies were directed and found that they predominantly recognized the N-terminal part of protein S. Finally we showed by thrombography a transient dramatic hypercoagulable state as a result of thrombin being unregulated by the dynamic protein C inhibitory system: in vitro thrombin generation, in response to a low concentration of tissue factor, was almost insensitive to activated protein C up to 25 nmol L )1 on day 4 while it was normally sensitive on day 42. For the first time, we demonstrated a temporal relationship between protein S deficiency, antibodies to protein S and hypercoagulability, thus supporting the pathogenic role of these antibodies.

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Section: Discussionmentioning

confidence: 99%

“…Peyvandi et al . also observed PS deficiency, presumably autoimmune, in a patient with deep vein thrombosis after varicella [6]. Josephson et al .…”

Section: Introductionmentioning

confidence: 98%

See 1 more Smart Citation

Anti‐protein S antibodies following a varicella infection: detection, characterization and influence on thrombin generation

Régnault

Boehlen²,

Özşahin

et al. 2005

Journal of Thrombosis and Haemostasis

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“…Protein S, protein C, anti thrombin III, factor V, antiphospholipid antibodies and homocystein levels can be measured. Peyvandi et al [16] described autoim- mune protein S deficiency and DVT after an attack of chickenpox. Nguyên et al [17] reported six cases of protein S deficiency secondary to varicella.…”

Section: Discussionmentioning

confidence: 99%

Rare complications of chickenpox in children

Sharma

Jora

et al. 2015

J Pediatr Infect Dis

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Chickenpox, caused by varicella-zoster virus, is a common infection of childhood, especially in developing countries like India, where immunization coverage is low. Fifty percent of children are infected by 5 years of age and 90% by 12 years of age. Though the disease usually runs a benign, self limited course, yet in severe infections life threatening complications may develop. Deep vein thrombosis and spreading infective necrotizing fascitis are very rare complications of this disease. We report here two cases of surgical emergencies associated with varicella and review the literature.

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“…The response of the sample is recorded as time required to induce a full occlusion of the aperture, which is expressed in terms of closure time (CT). The PFA-100 has been proposed for the screening of some inherited and acquired bleeding disorders, mostly von Willebrand factor (VWF) disease (1), qualitative and quantitative defects of platelets (2). Due to the unreliability of physical examination, personal history and bleeding time in children (3), PFA-100 might result useful for the screening of von Willebrand disease in paediatrics (4,5).…”

Section: Dear Sirmentioning

confidence: 99%