Clnical Aspects of Coagulation and Hemostasis I
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| 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
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| What is the inheritance pattern of Osler-Weber-Rendu Hereditary Hemorrhagic Telangiectasia disease? | Autosomal Dominant
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| What is the pathology of Osler-Weber-Rendu Hereditary Hemorrhagic Telangiectasia disease? | Telangiectases on tongue, face, mucous membranes, liver, and brain.
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| What is the most common inherited bleeding disorder? | von Willebrand Disease. Found in 1% of the population
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| What is the inheritance pattern of von Willebrand Disease? | Autosomal Dominant
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| What is the pathogenesis of Type I von Willebrand Disease? | Decrease von Willebrand Factor
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| What is the pathogenesis of Type II von Willebrand Disease? | Defective von Willebrand Factor
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| What is the pathogenesis of Type III von Willebrand Disease? | 2 defective genes for von Willebrand Factor resulting in the absence of VWF
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| What are the clinical manifestations of von Willebrand Disease? | Mucosal bleeding, epistaxis, bruising, surgical bleeding, and menorrhagia
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| What clotting factor is found on von Willebrand Factor? | FVIII
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| 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
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| 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
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| (T or F) vWF is an acute phase reactant and levels may be elevated in the setting of inflammation. | True.
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| Which ABO type has the highest level of vWF? | Type AB group
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| Which ABO type has the lowest level of vWF? | Type O group
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| What is the first line therapy for von Willebrand disease? | Desmopressin
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| What is the mechanism of desmopressin in von Willebrand disease? | Releases vWF stores from endothelium
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| What is Amicar? | Used for treatment of von Willebrand disease. It is an antifibrinolytic - stabilizes clot once formed.
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| What treatment option is available for Desmopressin non-responders in von | (blank)
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| What enzymes in the vitamin K pathway are inhibited by warfarin? | 1) Vit K reductase 2) Vit K epoxide reductase
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| What is the inheritance pattern of Hemophilia A? | X-linked recessive: males are affected and females are carriers
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| What is the inheritance pattern of Hemophilia B? | X-linked recessive: males are affected and females are carriers
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| Which clotting factor is defective or decreased in Hemophilia A? | Factor VIII
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| Which clotting factor is defective or decreased in Hemophilia B? | Factor IX
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| Which clotting factor is defective or decreased in Hemophilia Cq? | Factor XI
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| What is the inheritance pattern of Hemophilia C? | Autosomal recessive
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| Description of hemophilia classified as mild | Very rare sponaneous bleeding. Bleeding only occurs with moderate trauma.
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| Description of hemophilia classified as moderate | Unusual spontaneous bleeding. Bleeding occurs with minor trauma.
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| Description of hemophilia classified as severe | Common spontaneous bleeding. Delayed, excessive bleeding occurs iwth minimal trauma.
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| What are the major clinical features of Hemophilia? | Prolonged bleeding into joints, muscle, and soft tissue.
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| A disease process resulting from a burst of thrombin generation from endothelial cell injury or stimulation of tissue factor. | DIC
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| 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
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| What clotting factor is most sensitive to liver pathology resulting in a coagulopathy? | Factor VII
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| What is contained in cryoprecipitate? | vWF, Factor VIII, FActor XIII, Fibrinogen, Vitronectin, Fibronectin
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| In a mixing study, what is the minimum level of clotting factor needed for normal aPTT? | 30-40% of clotting factor
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