Low factor V level ameliorates bleeding diathesis in patients with combined deficiency of factor V and factor VIII-Reference-Cited by-同舟云学术

Low factor V level ameliorates bleeding diathesis in patients with combined deficiency of factor V and factor VIII

Published:2019-09-26 Issue:20 Volume:134 Page:1745-1754
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Shao Yanyan¹,Wu Wenman²,Xu Guanqun³,Wang Xuefeng³⁴,Ding Qiulan³⁴

Affiliation:

1. State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology,

2. Faculty of Medical Laboratory Science, and

3. Department of Laboratory Medicine, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China; and

4. Collaborative Innovation Center of Hematology, Shanghai Jiaotong University School of Medicine, Shanghai, China

Abstract

Combined factor V and factor VIII deficiency is a rare disorder associated with relatively mild bleeding diathesis. Shao and colleagues elucidate the double role of factor V as both a pro- and anticoagulant protein, demonstrating that decreased factor V may ameliorate factor VIII deficiency through decreasing the level of tissue factor pathway inhibitor.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/134/20/1745/1506084/blood886069.pdf

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4. Mutations in the ER-Golgi intermediate compartment protein ERGIC-53 cause combined deficiency of coagulation factors V and VIII;Nichols;Cell,1998

5. Clinical manifestations of combined factor V and VIII deficiency: a series of 37 cases from a single center in India;Viswabandya;Am J Hematol,2010

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