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H1N1 influenza (swine flu)-associated thrombotic microangiopathy with a markedly high plasma ratio of von Willebrand factor to ADAMTS13.

Identifieur interne : 000442 ( Ncbi/Curation ); précédent : 000441; suivant : 000443

H1N1 influenza (swine flu)-associated thrombotic microangiopathy with a markedly high plasma ratio of von Willebrand factor to ADAMTS13.

Auteurs : Rui Akiyama [Japon] ; Isao Komori ; Ryugo Hiramoto ; Ayami Isonishi ; Masanori Matsumoto ; Yoshihiro Fujimura

Source :

RBID : pubmed:21422695

Descripteurs français

English descriptors

Abstract

We describe an 18-year-old woman infected with H1N1 influenza followed by thrombotic microangiopathy. During the acute phase, her plasma levels of von Willebrand factor (VWF) were remarkably elevated, whereas those of ADAMTS13 were reduced without its inhibitors, generating a markedly high ratio of VWF to ADAMTS13 in circulation. A retrospective analysis established the following hypothesis: an influenza-mediated cytokine storm induced an enhanced release of unusually large VWF multimers (UL-VWFM) from vascular endothelial cells, generating platelet thrombi in microcirculatures under high shear stress. Plasma exchange removed UL-VWFM and cytokines, and rescued her life. This report sheds a light on a hitherto unrecognized influenza complication.

DOI: 10.2169/internalmedicine.50.4620
PubMed: 21422695

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pubmed:21422695

Le document en format XML

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<nlm:affiliation>Department of Pediatrics, Matsudo City Hospital Children's Medical Centre, Japan.</nlm:affiliation>
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<term>ADAM Proteins (blood)</term>
<term>ADAMTS13 Protein</term>
<term>Adolescent</term>
<term>Female</term>
<term>Humans</term>
<term>Influenza A Virus, H1N1 Subtype</term>
<term>Influenza, Human (blood)</term>
<term>Influenza, Human (complications)</term>
<term>Influenza, Human (diagnosis)</term>
<term>Retrospective Studies</term>
<term>Thrombotic Microangiopathies (blood)</term>
<term>Thrombotic Microangiopathies (diagnosis)</term>
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<term>Grippe humaine ()</term>
<term>Grippe humaine (diagnostic)</term>
<term>Grippe humaine (sang)</term>
<term>Humains</term>
<term>Microangiopathies thrombotiques (diagnostic)</term>
<term>Microangiopathies thrombotiques (sang)</term>
<term>Microangiopathies thrombotiques (étiologie)</term>
<term>Protéine ADAMTS13</term>
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<term>Protéines ADAM (sang)</term>
<term>Sous-type H1N1 du virus de la grippe A</term>
<term>Études rétrospectives</term>
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<term>ADAM Proteins</term>
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<term>von Willebrand Factor</term>
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<keywords scheme="MESH" type="chemical" qualifier="blood" xml:lang="en">
<term>ADAM Proteins</term>
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<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en">
<term>von Willebrand Factor</term>
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<term>ADAMTS13 Protein</term>
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<term>Protéines ADAM</term>
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<term>Facteur de von Willebrand</term>
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<term>Influenza, Human</term>
<term>Thrombotic Microangiopathies</term>
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<term>Influenza, Human</term>
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<term>Influenza, Human</term>
<term>Thrombotic Microangiopathies</term>
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<term>Grippe humaine</term>
<term>Microangiopathies thrombotiques</term>
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<term>Thrombotic Microangiopathies</term>
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<term>Facteur de von Willebrand</term>
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<term>Grippe humaine</term>
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<div type="abstract" xml:lang="en">We describe an 18-year-old woman infected with H1N1 influenza followed by thrombotic microangiopathy. During the acute phase, her plasma levels of von Willebrand factor (VWF) were remarkably elevated, whereas those of ADAMTS13 were reduced without its inhibitors, generating a markedly high ratio of VWF to ADAMTS13 in circulation. A retrospective analysis established the following hypothesis: an influenza-mediated cytokine storm induced an enhanced release of unusually large VWF multimers (UL-VWFM) from vascular endothelial cells, generating platelet thrombi in microcirculatures under high shear stress. Plasma exchange removed UL-VWFM and cytokines, and rescued her life. This report sheds a light on a hitherto unrecognized influenza complication.</div>
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