Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization

Haemophilia - Tập 10 Số 3 - Trang 218-231 - 2004
John Pasi1, Paul W. Collins2, David Keeling3, Simon Brown4, A. M. Cumming5, Gerard Dolan6, C. R. M. Hay5, F. G. H. Hill7, Michael Laffan8, I. R. Peake9
1Department of Haematology, Barts and The London, Queen Mary’s School of Medicine and Dentistry, London, UK
2Arthur Bloom Haemophilia Centre, University Hospital Wales, Cardiff, Wales
3Oxford Haemophilia Centre, Churchill Hospital, Oxford;
4Department of Haematology, Royal Free Hospital, London
5University Department of Haematology, Manchester Royal Infirmary, Manchester
6Department of Haematology, Queen's Medical Centre, Nottingham
7Department of Haematology, The Children's Hospital, Birmingham
8Department of Haematology, Imperial College, Hammersmith Hospital, London
9Division of Genomic Medicine, University of Sheffield, Sheffield, UK

Tóm tắt

Summary.  von Willebrand disease (VWD) is the commonest inherited bleeding disorder. The aim of therapy for VWD is to correct the two defects of haemostasis in this disorder, impaired primary haemostasis because of defective platelet adhesion and aggregation and impaired coagulation as a result of low levels of factor VIII. The objective of this guideline is to inform individuals making choices about the treatment and management of VWD including the use of therapeutic products. This is the second edition of this UK Haemophilia Centre Doctors' Organization (UKHCDO) guideline and supersedes the previous edition which was published in 1994.

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Tài liệu tham khảo

Sadler JE., 1994, A revised classification of von Willebrand disease, Thromb Haemost, 71, 520, 10.1055/s-0038-1642471

UK Haemophilia Centre Doctors’ Organisation von Willebrand Disease Working Party, 1997, Guidelines for the diagnosis and management of von Willebrand disease, Haemophilia, 3, 4

Mannucci PM, 1992, Desmopressin: a nontransfusional treatment of hemophilia and von Willebrand disease, Haemostasis, 22, 276

10.1111/j.1365-2141.1994.tb05127.x

De Sio L, 1985, Comparison between subcutaneous and intravenous DDAVP in mild and moderate hemophilia A, Thromb Haemost, 54, 387, 10.1055/s-0038-1657745

10.1055/s-0038-1646050

10.1007/BF01738476

10.1046/j.1365-2516.2000.00367.x

10.1056/NEJM198310063091402

10.1111/j.1600-0609.1990.tb00412.x

Casonato A, 1990, 1‐Desamino‐8‐D‐arginine vasopressin (DDAVP) infusion in type IIB von Willebrand's disease: shortening of bleeding time and induction of a variable pseudothrombocytopenia, Thromb Haemost, 64, 117, 10.1055/s-0038-1647265

10.1097/00001721-199412000-00013

Casonato A, 1999, Post‐DDAVP thrombocytopenia in type 2B von Willebrand disease is not associated with platelet consumption: failure to demonstrate glycocalicin increase or platelet activation, Thromb Haemost, 81, 224, 10.1055/s-0037-1614447

Fowler WE, 1989, DDAVP for type IIB von Willebrand disease, Blood, 74, 1859, 10.1182/blood.V74.5.1859.1859

10.1002/(SICI)1096-8652(199602)51:2<158::AID-AJH11>3.0.CO;2-E

10.1111/j.1365-2516.1996.tb00018.x

10.1056/NEJM198801143180215

10.1016/S0140-6736(89)92881-X

10.1016/0140-6736(90)92623-P

10.1016/S0140-6736(95)92560-0

10.1097/00006254-199807000-00025

10.1002/ajh.2830310310

10.1046/j.1365-2141.2000.01814.x

10.1159/000188524

British National Formulary.William Clowes Suffolk 2002;44:714.

Lindoff C, 1993, Treatment with tranexamic acid during pregnancy, and the risk of thromboembolic complications, Thromb Haemost, 70, 238, 10.1055/s-0038-1649475

Menache D, 1997, New treatments of von Willebrand disease: plasma derived von Willebrand factor concentrates, Thromb Haemost, 78, 566, 10.1055/s-0038-1657590

10.1002/ajh.2830250106

10.1111/j.1365-2141.1990.00234.x

10.1111/j.1365-2141.1990.tb02654.x

10.1046/j.1365-2516.1998.0040s3033.x

10.1182/blood.V79.12.3130.bloodjournal79123130

10.1046/j.1365-2516.1998.0040s3025.x

10.1055/s-0037-1613227

10.1055/s-0037-1613225

10.1046/j.1365-2516.1998.0040s3011.x

10.1111/j.1423-0410.1989.tb02031.x

Scharrer I, 1994, Experience with Haemate‐P in von Willebrand's disease in adults, Haemostasis, 24, 298

Kreuz W, 1994, Haemate‐P in children with von Willebrand's disease, Haemostasis, 24, 304

Lubetsky A, 1999, Safety and efficacy of continuous infusion of a combined factor VIII‐von Willebrand factor (vWF) concentrate (Haemate‐PTM) in patients with von Willebrand disease, Thromb Haemost, 81, 229, 10.1055/s-0037-1614448

10.1182/blood.V99.2.450

10.1046/j.1365-2516.2002.00688.x

10.1111/j.1423-0410.1987.tb04892.x

10.1111/j.1600-0609.1989.tb01244.x

10.1046/j.1365-2516.1998.0040s3048.x

10.1097/00001721-199701000-00002

10.1182/blood.V77.9.1901.1901

Sindet‐Pedersen S., 1991, Haemostasis in oral surgery–the possible pathogenetic implications of oral fibrinolysis on bleeding. Experimental and clinical studies of the haemostatic balance in the oral cavity, with particular reference to patients with acquired and congenital defects of the coagulation system, Dan Med Bull, 38, 427

10.1046/j.1365-2516.1999.00285.x

10.1002/(SICI)1096-8652(199612)53:4<234::AID-AJH4>3.0.CO;2-Z

10.1016/S0140-6736(97)08248-2

10.1136/bmj.316.7138.1122

10.1136/bmj.313.7057.579

10.1055/s-0038-1649813

10.1111/j.1471-0528.1995.tb11293.x

10.1046/j.1365-2516.1998.00147.x

Rodeghiero F, 1996, Prospective multicenter study on subcutaneous concentrated desmopressin for home treatment of patients with von Willebrand disease and mild or moderate hemophilia A, Thromb Haemost, 76, 692, 10.1055/s-0038-1650645

Kobrinsky N, 1997, Efficacy of Stimate (desmopressin acetate) nasal spray, 1.5 mg ml−1, for the treatment of menorrhagia in women with inherited bleeding disorders, Blood, 90, 106

10.1016/0029-7844(95)00062-V

10.1111/j.1365-2141.1987.00349.x

Hill FG., 1982, Haematological disorders, Clin Obstet Gynaecol, 9, 75, 10.1016/S0306-3356(21)00186-2

10.1055/s-0038-1649623

10.1016/S0140-6736(76)90729-7

10.1002/ajh.2830460325

10.1016/S0140-6736(02)08542-2

10.1016/S1590-8658(01)80087-6

Cattaneo M, 1989, DDAVP shortens the prolonged bleeding times of patients with severe von Willebrand disease treated with cryoprecipitate. Evidence for a mechanism of action independent of released von Willebrand factor, Blood, 74, 1972, 10.1182/blood.V74.6.1972.1972

10.1055/s-0038-1649942

Mannucci PM., 1997, Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years, Blood, 90, 2515, 10.1182/blood.V90.7.2515

Gralnick HR, 1986, DDAVP in type IIa von Willebrand's disease, Blood, 67, 465, 10.1182/blood.V67.2.465.465

Ciavarella N, 1996, Use of recombinant factor VIIa (NovoSeven) in the treatment of two patients with type III von Willebrand's disease and an inhibitor against von Willebrand factor, Haemostasis, 26, 150

10.1046/j.1365-2516.1999.0050s2028.x

Veyradier A, 2000, Willebrand Syndrome: from pathophysiology to management, Thromb Haemost, 4, 175, 10.1055/s-0037-1613993

AHCPR, 1992, Acute Pain Management: Operative or Medical Procedures and Trauma