Clinical and laboratory diagnosis of von Willebrand disease: A synopsis of the 2008 NHLBI/NIH guidelines

被引:72
|
作者
Nichols, William L. [1 ,2 ,3 ]
Rick, Margaret E. [4 ]
Ortel, Thomas L. [5 ,6 ]
Montgomery, Robert R. [7 ,8 ]
Sadler, J. Evan [9 ]
Yawn, Barbara P. [10 ,11 ]
James, Andra H. [12 ]
Hultin, Mae B. [13 ]
Manco-Johnson, Marilyn J. [14 ,15 ]
Weinstein, Mark [16 ]
机构
[1] Mayo Clin, Special Coagulat Lab, Coll Med, Dept Lab Med & Pathol,Div Hematopathol, Rochester, MN 55905 USA
[2] Mayo Clin, Coll Med, Div Hematol & Internal Med, Coagulat Clin, Rochester, MN 55905 USA
[3] Mayo Clin, Coll Med, Div Hematol & Internal Med, Comprehens Hemophilia Ctr, Rochester, MN 55905 USA
[4] NIH, Hematol Serv, Warren Grant Magnuson Clin Ctr, Bethesda, MD 20892 USA
[5] Duke Univ, Med Ctr, Dept Med, Div Hematol, Durham, NC 27710 USA
[6] Duke Univ, Med Ctr, Dept Pathol, Clin Coagulat Lab, Durham, NC 27710 USA
[7] Med Coll Wisconsin, Blood Ctr Wisconsin, Blood Res Inst, Milwaukee, WI 53226 USA
[8] Med Coll Wisconsin, Dept Pediat, Sect Pediat Hematol, Milwaukee, WI 53226 USA
[9] Washington Univ, Dept Med, St Louis, MO USA
[10] Olmsted Med Ctr, Dept Res, Rochester, MN USA
[11] Univ Minnesota, Dept Family & Community Med, Minneapolis, MN USA
[12] Duke Univ, Med Ctr, Dept Obstet & Gynecol, Durham, NC 27710 USA
[13] SUNY Stony Brook, Dept Med, Stony Brook, NY 11794 USA
[14] Univ Colorado Denver, Dept Pediat, Aurora, CO USA
[15] Childrens Hosp, Ctr Canc & Blood Disorders, Denver, CO 80218 USA
[16] US FDA, Ctr Biol Evaluat & Res, Off Blood Res & Review, Rockville, MD 20857 USA
关键词
CARDIOMYOPATHY;
D O I
10.1002/ajh.21405
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Von Willebrand factor (VWF) mediates blood platelet adhesion and accumulation at sites of blood vessel injury, and also carries coagulation factor VIII (FVIII) that is important for generating procoagulant activity. Von Willebrand disease (VWD), the most common inherited bleeding disorder, affects males and females, and reflects deficiency or defects of VWF that may also cause decreased FVIII. It may also occur less commonly as an acquired disorder (acquired von Willebrand syndrome). This article briefly summarizes selected features of the March 2008 evidence-based clinical and laboratory diagnostic recommendations from the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel for assessment for VWD or other bleeding disorders or risks. Management of VWD is also addressed in the NHLBI guidelines, but is not summarized here. The VWD guidelines are available at the NHLBI Web site (http://www.nhlbi.nih.gov/guidelines/ vwd). Am. J. Hematol. 84:366-370, 2009. (C) 2009 Wiley-Liss, Inc.
引用
收藏
页码:366 / 370
页数:5
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