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Lecture 30

Clnical Aspects of Coagulation and Hemostasis I

QuestionAnswer
Clinical manifestations of primary hemostatic defects 1)Superficial purpura 2)Petechiae 3)Epistaxis 4)Mucous membrane bleeding 5)Prolonged immediate bleeding 6)"oozy-bruisy" paitent
What is the inheritance pattern of Osler-Weber-Rendu Hereditary Hemorrhagic Telangiectasia disease? Autosomal Dominant
What is the pathology of Osler-Weber-Rendu Hereditary Hemorrhagic Telangiectasia disease? Telangiectases on tongue, face, mucous membranes, liver, and brain.
What is the most common inherited bleeding disorder? von Willebrand Disease. Found in 1% of the population
What is the inheritance pattern of von Willebrand Disease? Autosomal Dominant
What is the pathogenesis of Type I von Willebrand Disease? Decrease von Willebrand Factor
What is the pathogenesis of Type II von Willebrand Disease? Defective von Willebrand Factor
What is the pathogenesis of Type III von Willebrand Disease? 2 defective genes for von Willebrand Factor resulting in the absence of VWF
What are the clinical manifestations of von Willebrand Disease? Mucosal bleeding, epistaxis, bruising, surgical bleeding, and menorrhagia
What clotting factor is found on von Willebrand Factor? FVIII
What laboratory tests are part of the workup of von Willebrand disease? Detection of VWF Ag, VWF risocetin activity, measurement of multimers levels, measurement of FVIII levels
What variant of von Willebrand disease would demonstrate discordance between the VWF antigen level and activity level? Type 2 defect - disease resulting from defective vWF
(T or F) vWF is an acute phase reactant and levels may be elevated in the setting of inflammation. True.
Which ABO type has the highest level of vWF? Type AB group
Which ABO type has the lowest level of vWF? Type O group
What is the first line therapy for von Willebrand disease? Desmopressin
What is the mechanism of desmopressin in von Willebrand disease? Releases vWF stores from endothelium
What is Amicar? Used for treatment of von Willebrand disease. It is an antifibrinolytic - stabilizes clot once formed.
What treatment option is available for Desmopressin non-responders in von (blank)
What enzymes in the vitamin K pathway are inhibited by warfarin? 1) Vit K reductase 2) Vit K epoxide reductase
What is the inheritance pattern of Hemophilia A? X-linked recessive: males are affected and females are carriers
What is the inheritance pattern of Hemophilia B? X-linked recessive: males are affected and females are carriers
Which clotting factor is defective or decreased in Hemophilia A? Factor VIII
Which clotting factor is defective or decreased in Hemophilia B? Factor IX
Which clotting factor is defective or decreased in Hemophilia Cq? Factor XI
What is the inheritance pattern of Hemophilia C? Autosomal recessive
Description of hemophilia classified as mild Very rare sponaneous bleeding. Bleeding only occurs with moderate trauma.
Description of hemophilia classified as moderate Unusual spontaneous bleeding. Bleeding occurs with minor trauma.
Description of hemophilia classified as severe Common spontaneous bleeding. Delayed, excessive bleeding occurs iwth minimal trauma.
What are the major clinical features of Hemophilia? Prolonged bleeding into joints, muscle, and soft tissue.
A disease process resulting from a burst of thrombin generation from endothelial cell injury or stimulation of tissue factor. DIC
What are the clinical features of DIC? 1)Thrombosis of small vessels cause end organ damage 2)Fibrinolysis of thrombus cause reperfusion injury 3)Consumption of platelets and clotting factors cause bleeding
What clotting factor is most sensitive to liver pathology resulting in a coagulopathy? Factor VII
What is contained in cryoprecipitate? vWF, Factor VIII, FActor XIII, Fibrinogen, Vitronectin, Fibronectin
In a mixing study, what is the minimum level of clotting factor needed for normal aPTT? 30-40% of clotting factor
Created by: UVAPATH4