Von Willebrand Disease
DOI:
https://doi.org/10.61427/jcpr.v1.i1.2021.9Keywords:
Desmopressin, Von Willebrand Disease, Von Willebrand FactorAbstract
Von Willebrand Disease (VWD) is a disorder of blood in which the proper clotting of the blood does not occur. Von Willebrand Factor (VMF) is an important multimeric glycoprotein of the blood that helps to stop the bleeding. When the function of this von Willebrand factor is impaired we can observe this Von Willebrand Disease. Usually, the VWF in the blood attaches to small blood cells called platelets when a person is injured and starts to bleed. In order to stop bleeding at the site of injury, this VWF helps the platelets stick together to form a clot by acting like a glue. In case of the patients with Von Willebrand Disease it is very difficult to stop the bleeding and is often life threatening. In this review, the symptoms, diagnosis and the treatment strategies were discussed. Health care professionals expertise in the haemostasis are required in the clinical scenario for better management of this disease. As it is an inherited disease, special care must be taken in evaluating the family history during the diagnosis and extensive research is still required for providing the better management and treatment.
Downloads
References
Bharati KP, Prashanth UR. Von Willebrand disease: an overview. Indian J Pharm Sci. 2011; 73(1): 7-16.
Sadler JE. A revised classification of von Willebrand disease. For the subcommittee on von Willebrand Factor of the scientific and standardization committee of the International society on thrombosis andhaemostasis. Thromb Haemost. 1994; 71: 520-5.
Kadir RA, Chi C. Women and von Willebrand disease: Controversies in diagnosis and management. Semin Thromb Hemost. 2006; 32: 605-15.
James P, Lillicrap D. Genetic testing for von Willebrand disease: The Canadian experience. Semin Thromb Hemost. 2006; 32: 546-52.
Federici AB. Acquired von Willebrand syndrome: An underdiagnosed and misdiagnosed bleedingcomplication in patients with lymphoproliferative and myeloproliferative disorders. Semin Hematol.2006; 43: 48-58.
Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL. Von Willebrand disease (VWD): Evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHBLI) Expert panel report (USA). Haemophilia. 2008; 14: 171-232.
Quiroga T, Goycoolea M, Munoz B, Morales M, Aranda E, Panes O. Template bleeding time and PFA-100 have low sensitivity to screen patients with hereditary mucocutaneous hemorrhages: Comparative study in 148 patients. J Thromb Haemost. 2004; 2: 892-8.
Orstavik KH, Magnus P, Resiner H, Berg K, Graham JB, Nance W. Factor VIII and factor IX in a twin population: Evidence for a major effect of ABO locus on factor VIII level. Am J Hum Genet. 1985; 37: 89-101.
Pruthi RK. A practical approach to genetic testing for von Willebrand Disease. Mayo Clin Proc.2006; 81: 679-91.
Sadler JE, Rodeghiero F. ISTH SSC Subcommittee on von Willebrand Factor. Provisional criteria for the diagnosis of VWD type 1. J Thromb Haemost. 2005; 3: 775-7.
Kaufmann JE, Vischer UM. Cellular mechanisms of the haemostatic effects of Desmopressin (DDAVP). J Thromb Haemost. 2003; 1: 682-9.
Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A. 1-Deamino-8-d-arginine vasopressin: A new pharmacological approach to the management of haemophilia and von Willebrand's disease. Lancet.1977; 1: 869-72.
De La Fuente B, Kasper CK, Rickles FR, Hoyer LW. Response of patients with mild and moderate haemophilia A and von Willebrand's disease treatment with desmopressin. Ann Intern Med. 1985; 103: 6-14.
Agrawal YP, Dzik W. The VWF content of factor VIII concentrates. Transfusion. 2001; 41: 153-4.
Chang AC, Rick ME, Ross PL, Weinstein MJ. Summary of a workshop on potency and dosage of von Willebrand factor concentrates. Haemophilia. 1998; 4(3): 1-6.
Dobrkovska A, Krzensk U, Chediak JR. Pharmacokinetics, efficacy and safety of Humate-P in vonWillebrand disease. Haemophilia. 1998; 4(Suppl 3): 33-9.
Miller RA May MW, Hendry WF, Whitfield HN, Wickham JE. The prevention of secondary haemorrhage after prostatectomy: The value of antifibrinolytic therapy. Br J Urol. 1980; 52: 26-8.
Mannucci PM. Treatment of von Willebrand disease. N Eng J Med. 2004; 351: 683-94.
Zwischenberger JB, Brunston RL, Jr, Swann JR, Conti VR. Comparision of two topical collagen-based haemostatic sponges during cardiothoracic procedures. J Invest Surg. 1999; 12: 101-6.
Federici AB, Sacco R, Stabile F, Carpenedo M, Zingaro E, Mannucci PM. Optimising local therapy during oral surgery in patients with von Willebrand disease: Effective results from a retrospective analysis of 63 cases. Haemophilia. 2000; 6: 71-7.
Rakocz M, Mazar A, Varon D, Spierer S, Blinder D, Martinowitz U. Dental extractions in patients with bleeding disorders. The use of fibrin glue. Oral Surg Oral Med Oral Pathol. 1993; 75: 280-2.
Published
How to Cite
Issue
Section
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Most read articles by the same author(s)
- Hima Bindu Mylapalli, Covid Tongue - A New Symptom of Covid-19 , Journal of Clinical and Pharmaceutical Research: JCPR Volume:1/Issue:2 (2021)
- Sireesha Maram, T Venkataramana Reddy, Hima Bindu Mylapalli, Sudeepthi Padala, Hemanth Kumar Vangala, Aksha Susmitha Jangam, Behcet’s Disease: A Multifaceted Affliction , Journal of Clinical and Pharmaceutical Research: JCPR Volume:1/Issue:3 (2021)
.