Presentation
Acronym VWDP Synonyms BDPLT3 Bleeding disorder platelet-type 3 Pseudo-vWD von Willebrand disease platelet-type Disclaimer Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. [uniprot.org]
[…] type-von Willebrand disease Pseudo-von Willebrand disease type 2B Prevalence: <1 / 1 000 000 Inheritance: Autosomal dominant Age of onset: - ICD-10: D69.8 OMIM: 177820 UMLS: C1280798 MeSH: - GARD: 8312 MedDRA: - The documents contained in this web site are presented [orpha.net]
Hereditary deficit of the release reaction: 1. with absent granulations (grey platelet syndrome) 2. with present granulations and activation abnormalities. Aggregation to ADP, collagen, arachidonic acid, and ristocetin 1. [medichub.ro]
Entire Body System
- Anemia
Angioedema Angioimmunoblastic T-Cell Lymphoma Angioma Serpiginosum Angiomatosis Angiosarcoma Aniridia 1 Annular Erythema Anorexia Nervosa 1 Anterior Uveitis Antiphospholipid Syndrome Antisynthetase Syndrome Antithrombin Iii Deficiency Anuria Aplastic Anemia [eutopia.school]
Cardiovascular
- Hypertension
Early-Onset, Autosomal Dominant, with Severe Exacerbation in Pregnancy Hypertensive Encephalopathy Hyperthyroidism Hypoascorbemia Hypoglycemia Idiopathic Neutropenia Iga Glomerulonephritis Igg4-Related Disease Ileitis Immune System Disease Immunoglobulin [eutopia.school]
Treatment
Treatment Treatment for VWD depends on the diagnosis and severity. The mainstay of treatment is DDAVP (desmopressin acetate), the synthetic version of a natural hormone vasopressin. [hemophilia.org]
You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments. [rarediseases.info.nih.gov]
Prognosis
Bleeding Assessment Tools: Limits and Advantages for the Diagnosis and Prognosis of Inherited Bleeding Disorders. Semin Thromb Hemost. 2016 Jul. 42 (5):463-70. Gill JC, Castaman G, Windyga J, Kouides P, Ragni M, Leebeek FW, et al. [labtestsonline.it]
Epidemiology
Inconsistency of association between type 1 von Willebrand disease phenotype and genotype in families identified in an epidemiological investigation. Thromb Haemost, 82 (1999), pp. 1065-1070 [21] E.J. Favaloro. [elsevier.es]
Pathophysiology
Journal N Engl J Med 306:326-33 (1982) DOI:10.1056/NEJM198202113060603 Reference PMID:23934752 Authors Othman M, Kaur H, Emsley J Title Platelet-type von Willebrand disease: new insights into the molecular pathophysiology of a unique platelet defect. [genome.jp]
Prevention
Aminocaproic acid and tranexamic acid are antifibrinolytics agents that prevent the breakdown of blood clots. These drugs are often recommended before dental procedures, to treat nose and mouth bleeds, and for menorrhagia. [hemophilia.org]
[…] evidence provides precise information on the mechanisms of the disease and the risk of bleeding associated with the deficiency or abnormality of the VWF.43Treatment The treatment depends on the subtype of the von Willebrand disease; however, therapies to prevent [elsevier.es]