کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2106521 1083521 2011 7 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Platelet-type Von Willebrand disease: Three decades in the life of a rare bleeding disorder
کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی تحقیقات سرطان
پیش نمایش صفحه اول مقاله
Platelet-type Von Willebrand disease: Three decades in the life of a rare bleeding disorder
چکیده انگلیسی

Almost thirty years has passed since the description of platelet-type von Willebrand disease. Named initially pseudo-von Willebrand disease, this mild mucocutaneous bleeding disorder has an intrinsic defect in platelets rather than VWF. Due to its unique features and its similarity to the more common type 2B VWD, many investigators were intrigued by its nature, pathophysiology and molecular genetics aspects. The challenges to diagnose this disorder have created debate in literature about the best method of discrimination from type 2B VWD. The recent development of the PT-VWD mouse model carries huge potential for further understanding of the disease as well as the platelet defect and its influence on other biological processes. This review provides a basic as well as updated knowledge about the PT-VWD including history, clinical and laboratory features and treatment in addition to the diagnostic challenges with a focus on molecular genetics aspects and PT-VWD research.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Blood Reviews - Volume 25, Issue 4, July 2011, Pages 147–153
نویسندگان
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