Postinfection Purpura Fulminans in a Patient Heterozygous for Prothrombin G20210a and Acquired Protein S Resistance

Al‐Ismail, Saad; Collins, Peter William; Najib, Riayd; James-Ellison, M.; O'Hagan, M

doi:10.1080/088800199276877

Cited by 21 publications

(13 citation statements)

References 6 publications

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“…12 The clinical manifestations can also go along with streptococcal coinfection, 12,15 with the simultaneous presence of procoagulant antiphospholipid antibodies 13,16 and/or a concomitant thrombophilic disorder. 17 A severe PS deficiency, failing to support activated PC, allows an uncontrolled and excessive production of thrombin, leading to widespread and systemic intravascular fibrin deposition, that is, DIC. This has been demonstrated in patients with postinfectious PF who were showing increased plasma levels of prothrombin fragments 1+2 12,13 and in vitro by showing an acquired resistance to activated PC in the presence of anti-PS antibodies.…”

Section: Discussionmentioning

confidence: 99%

Chickenpox Is Not Always Benign

Fluri¹,

Kaczala²,

Leibundgut³

et al. 2010

Pediatric Emergency Care

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We present 2 patients, who were admitted owing to rapidly progressing purpuric lesions due to postvaricella purpura fulminans, a coagulopathy leading to life- or limb-threatening thrombosis caused by a severe transient autoimmune protein S deficiency. Laboratory results were being consistent with disseminated intravascular coagulation secondary to protein S deficiency; treatment with fresh frozen plasma, intravenous immunoglobulins, and prednisone was started. In our experience, a prompt therapy may limit the course and the extent of the disease. We present a review of the topic with supporting literature for the therapeutic options. Therefore, we should be reminded that purpura fulminans is a rare but severe complication of chickenpox, which demands quick action.

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Section: Discussionmentioning

confidence: 99%

Chickenpox Is Not Always Benign

Fluri¹,

Kaczala²,

Leibundgut³

et al. 2010

Pediatric Emergency Care

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“…Anecdotal reports have indicated that the presence of congenital thrombophilia may exacerbate the coagulopathy associated with severe infection and may even result in purpura fulminans. [55][56][57][58][59] Indeed, various coagulation defects seem to be associated with an aggravated coagulation response to infectious agents or sepsis, although a systematic overview is missing. 60,61 Experimental studies seem to confirm the hypothesis.…”

Section: Prothrombotic Mechanisms In Sepsismentioning

confidence: 99%

Sepsis and Thrombosis

Levi

Schultz

Poll

2013

Semin Thromb Hemost

171

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Activation of coagulation frequently occurs in severe infection and sepsis and may contribute to the development of thrombosis. Coagulation abnormalities in sepsis range from a small decrease in platelet count and subclinical prolongation of global clotting times to fulminant disseminated intravascular coagulation (DIC), characterized by simultaneous widespread microvascular thrombosis and profuse bleeding from various sites. Septic patients with severe forms of DIC may present with manifest thromboembolic disease or clinically less apparent microvascular fibrin deposition, which predominantly presents as multiple organ dysfunction. Thrombophilia is associated with a prohemostatic state and consequently with an increased tendency to develop thrombosis. Hypothetically, patients with thrombophilia may suffer from more severe coagulopathy in case of severe infection or sepsis, which may result in a more serious clinical course and an unfavorable outcome. On the basis of the knowledge of the pathogenesis of thrombosis in severe inflammation and sepsis, strategies aimed at the inhibition of coagulation activation have been developed and have been found favorable in experimental and clinical studies.

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“…Finally, characterization of epitopes on PS was reported by one team [7]. Anti‐PS antibodies have also been found in children with purpura fulminans after infections other than varicella [8,9].…”

Section: Introductionmentioning

confidence: 99%

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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Postinfection Purpura Fulminans in a Patient Heterozygous for Prothrombin G20210a and Acquired Protein S Resistance

Cited by 21 publications

References 6 publications

Chickenpox Is Not Always Benign

Chickenpox Is Not Always Benign

Sepsis and Thrombosis

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

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