Clinical and Applied Thrombosis Discussion Essay
Von willenbrand Disease
It is an inherited genetic disorder bleeding disorder that results from dysfunction or deficiency of the von Willebrand factor (Castaman & Linari, 2017). The interaction between platelets and the blood vessel wall hinders primary hemostasis in the defective von Willebrand factor.
Pathophysiology
Von Willebrand factor is a multimer produced by endothelial cells and megakaryocytes. The size of the multimer influences the physiological hemostatic effect (Garmo, 2022). In most occasions, when a person gets injured and starts to bleed, the VWF attaches to platelets to facilitate the process of clot formation. In people with von Willebrand, the clot might take longer to form, resulting in bleeding problems. Clinical and Applied Thrombosis Discussion Essay
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Clinical presentation
Majority of patients present with bleeding problems such as excessive and recurrent bruising, prolonged bleeding from mucosal surfaces (menstruation, epistaxis, dental extractions), and prolonged bleeding from minor skin trauma (Arjun, 2017). On examination, there are signs of bruising or bleeding include; hematomas and petechiae. In other instances, the disease may imitate hemophilia and is present mainly with bleeding into joints, soft tissues, and hematuria.
Diagnosis
- Full hemogram: Check the platelet count.
- Von Willebrand factor antigen: Tests for the quantitative defect which measures the von Willebrand factor protein levels (Leebeek & Eikenboom, 2016).
- Coagulation profile: To check the activated partial thromboplastin time, which evaluates and measures the clotting factors.
- Von Willebrand factor activity is a qualitative analysis of the von Willebrand factor’s physiological function.
Treatment
- Type 1: Desmopressin is the first line treatment of choice for people von Willebrand.
- Type 2: Patients respond variably to desmopressin and thus are given von Willebrand factor concentrates
- Type 3: The most preferred treatment is recombinant von Willebrand factor or virus-inactivated and recombinant–containing FVIII concentrates. Clinical and Applied Thrombosis Discussion Essay
References
Arjun. (2017). von Willebrand Disease: A Concise Review and Update for the Practicing Physician – Arjun Swami, Varinder Kaur, 2017. Clinical and Applied Thrombosis/Hemostasis. https://journals.sagepub.com/doi/10.1177/1076029616675969
Castaman, G., & Linari, S. (2017). Diagnosis and Treatment of von Willebrand Disease and Rare Bleeding Disorders. Journal of Clinical Medicine, 6(4), 45. https://doi.org/10.3390/jcm6040045
Garmo. (2022, September 5). Physiology, Clotting Mechanism. https://pubmed.ncbi.nlm.nih.gov/29939572/
Leebeek, F. W. G., & Eikenboom, J. C. J. (2016). Von Willebrand’s Disease. New England Journal of Medicine, 375(21), 2067–2080. https://doi.org/10.1056/nejmra1601561 Clinical and Applied Thrombosis Discussion Essay