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

被引：15

作者：

Akiyama, Rui ^{[1
]}

Komori, Isao ^{[1
]}

Hiramoto, Ryugo ^{[1
]}

Isonishi, Ayami ^{[2
]}

Matsumoto, Masanori ^{[2
]}

Fujimura, Yoshihiro ^{[2
]}

机构：

[1] Matsudo City Hosp, Childrens Med Ctr, Dept Pediat, Matsudo, Chiba, Japan

[2] Nara Med Univ, Dept Blood Transfus Med, Nara, Japan

来源：

INTERNAL MEDICINE | 2011年 / 50卷 / 06期

关键词：

swine influenza; TMA; cytokine storm; UL-VWFM; ADAMTS13; DISSEMINATED INTRAVASCULAR COAGULATION; THROMBOCYTOPENIC PURPURA; INFECTION;

D O I：

10.2169/internalmedicine.50.4620

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

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.

引用

页码：643 / 647

页数：5

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