Pathology - Von Willebrand Disease
Von Willebrand disease is an autosomal dominant disorder characterized by a deficit in von Willebrand factor (vWF). Acquired von Willebrand disease is linked to cancer and autoimmune disorders, and is caused by reduced production and greater elimination of vWF. Von Willebrand disease is the prevailing genetic bleeding illness, impacting around 1% of the global population. Study of diseases and their effects on the body. The absence of von Willebrand factor (vWF) leads to a decrease in platelet attachment to the inner lining of blood vessels during damage, which in turn leads to a reduced production of platelet plugs. Deficiency of von Willebrand factor (vWF) leads to a functional deficiency of factor VIII, as vWF also serves as a carrier protein for factor VIII. This deficiency impairs the intrinsic pathway of coagulation. Symptoms and signs Epistaxis; ecchymosis Laboratory results: The patient exhibits an extended activated partial thromboplastin time (PTT) and prolonged bleeding time, but their prothrombin time (PT) and thrombin time are within normal range. Additionally, their platelet count is normal. Therapy Refrain from using aspirin and other blood thinners; consider desmopressin or factor VIII replacement if needed. There are two bleeding disorders caused by deficiencies in platelet aggregation: (1) Glanzmann thrombasthenia, caused by a deficiency of glycoproteins Ilb and IIIa, which act as receptors for fibrinogen, and (2) Bernard-Soulier disease, caused by a deficiency of glycoprotein Ib, which acts as a receptor for vWF factor. Both illnesses exhibit mucosal bleeding, an extended bleeding period, and normal partial thromboplastin time (PTT) and prothrombin time (PT).
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