☆ 4.2 Article

H1N1 Influenza (Swine Flu)-Associated Thrombotic Microangiopathy with a Markedly High Plasma Ratio of von Willebrand Factor to ADAMTS13

INTERNAL MEDICINE (2011)

期刊

INTERNAL MEDICINE

卷 50, 期 6, 页码 643-647

出版社

JAPAN SOC INTERNAL MEDICINE

DOI: 10.2169/internalmedicine.50.4620

关键词

swine influenza; TMA; cytokine storm; UL-VWFM; ADAMTS13

类别

Medicine, General & Internal

资金

Ministry of Education, Culture, Sports, Science and Technology of Japan
Ministry of Health, Labor, and Welfare of Japan

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Protocol

Reagent

摘要

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.

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