Von Willebrand disease - detection, diagnostics and treatment
نویسندگان
چکیده
von Willebrand disease is the most common inherited bleeding disorder, with mucocutaneous and menorrhagia as leading clinical manifestations. Cause of diathesis deficit or dysfunction vonWillebrand factor, plasma protein important roles in adhesion platelets to site vascular injury transport protection coagulation factor VIII. Prevalance disease, according different registries, between 1 on 100 persons 10000 persons. These data shows that detection disorder not easy many cases are undiagnosed. That why assessment tools developed they widely used for years. Confirmation diagnosis through laboratory testing. Some tests easily accessible. Therapy depends goals treatment (stopping bleeding, prophylaxis preoperative management). Last year, new guideline diagnostics was published by experts World Federation Haemophilia, International Society Thrombosis Haemostasis, American Hematology National Hemophilia Foundation. In this paper, recommendations detection, presented.
منابع مشابه
Treatment of patients with von Willebrand disease
Von Willebrand disease (vWD) is the most common hereditary bleeding disorder. The aim of therapy is to correct the dual hemostatic defect, due to defective platelet adhesion-aggregation and abnormal coagulation due to Factor VIII (FVIII) deficiency. The choice of treatment depends on a number of factors, including the severity of the bleed, the procedure planned, the subtype and severity of the...
متن کاملDefining von Willebrand disease.
for repeat testing. The authors’ conclusion that it should be considered for screening patients requires further consideration and study but conceivably, this assay should improve the future of VWD diagnosis. Conflict-of-interest disclosure: P.D.J. has received research funding from Bayer, CSL Behring, and Octapharma; and honoraria for educational talks from Baxalta, CSL Behring, and Octapharma. n
متن کاملVon Willebrand disease
hereditary elliptocytosis and hereditary pyropoikilocytosis. Blood Cells Mol Dis 1996;22:254–8. 8 Delaunay J. The hereditary stomatocytoses: genetic disorders of the red cell membrane permeability to monovalent cations. Semin Hematol 2004;41:165–72. 9 Carella M, Stewart G, Ajetunmobi JF et al. Genomewide search for dehydrated hereditary stomatocytosis (hereditary xerocytosis): mapping of locus ...
متن کاملVon Willebrand disease
There are three major types of VWD disease. Type 1, the most frequent form, is characterized by a partial quantitative deficiency in von Willebrand factor (VWF). Type 2 is a qualitative deficiency, and Type 3 is a virtually complete deficiency. Type 2 VWD is divided into four subtypes. Type 2A includes variants with decreased platelet adhesion caused by a selective deficiency in high-molecular ...
متن کاملVon Willebrand disease
There are three major types of VWD disease. Type 1, the most frequent form, is characterized by a partial quantitative deficiency in von Willebrand factor (VWF). Type 2 is a qualitative deficiency, and Type 3 is a virtually complete deficiency. Type 2 VWD is divided into four subtypes. Type 2A includes variants with decreased platelet adhesion caused by a selective deficiency in high-molecular ...
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ژورنال
عنوان ژورنال: Medicinski Pregled
سال: 2022
ISSN: ['0025-8105', '1820-7383']
DOI: https://doi.org/10.2298/mpns22s1147r