Tbl - Blood And Lymph - Bleeding Disorders (Acquired Thrombophilias, Bleeding And Bruising Disorders)

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Hemophilia A

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VIII manufactured by endothelial cells and megakaryocytes. (inc Factor VIII in liver disease). Inactivated by Protein Cand activated by F X. Hemorrhage, joints, body cavities and muscles. Dec. IX –VIII complex - dec fibrin and thrombin production  decrease TAFI (thrombin activated fibrinolysis inhibitor)  inc fibrinolysis  increased bleeding.

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Hemophilia B

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IX Christmas disease, Vit K dependent, Liver manufactured, Hemearthroses, subcutaneous bleeds, and CNS bleeds

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Hemophilia C

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XI Ashkenazi Jewish families

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How to treat Bleeding of Hemophilia A

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Novo 7 (recombinant activated F VII)

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Factor XIII deficiency

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caused by liver disease… umbilical stump and intracranial bleeding.

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Factor XIII Dx via

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urea solubility test.

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aPTT or PT goes from abnormal in the patient to normal in the mix (50% of normal factor from normal plasma). This indicates

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factor deficiency

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aPTT or PT is abnormal in the patient and remains abnormal in the mix. This indicates

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inhibitors are present

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shortest t1/2 is associated with what factor?
where is it made? test results?

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Factor VII. Manufactured in the liver; vit K dependent (PT is prolonged before PTT is prolonged)

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Factor I

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liver & megakaryocytes; Deficiency à bleeding or asymptomatic.

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Dysfibrinogenemia is associated with

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Factor I. abnormal bleeding or clotting; repeated fetal loss. Thrombin time is abnormal in this condition

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Dysprothrombinemia is associated with

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Factor II (prothrombin). abnormal molecule resulting in deficiency. Testing – PT, aPTT & Ecarin clotting time (abnormal).

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Factor III deficiency

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acquired is common (warfarin adm. & vitamin K deficiency, liver disease). Made in liver; Vitamin K dependent

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Factor V is associated with

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liver, megakaryocytes and endothelial cells. Inactivation by Protein C, deficiency = bleeding

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Protein C (APC) is associated with

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liver; vit K dependent. Protein S is cofactor. Inactivates Va, VIIIa, and PAI-1. Defieciency: venous thrombosis and purpura fulminans (children. Gangrene).

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Warfarin-induced skin necrosis is associated with

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not enough heparin admin with warfarin. warfarin interferes with vit K… and Protein C and S are Vit K dependent… inc. superficial clotting in skin… skin necrosis.

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Free protein S decreased by

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increase of C4b .. acute phase reactant.  less Protein S to activate Protein C  inc F V and F VIII activity  thrombosis. (type III is involved with inc C4b).

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APC RESISTANCE

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receptor (ie Factor V Leiden) is defected.. thrombosis occurs (Va and VIIIa are not deactivated.

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Test for APC resistance

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RT PCR for F 5 Leiden, Prolonged procoagulant activity, APC resistant assay is abnormal

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Prothrombin Gene mutation G20210A leads to

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inc levels of prothrombin  venous thrombosis… and maybe arterial. Hetero = 3X risk of venous thrombosis.

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Testing for Prothrombin Gene mutation

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RT PCR///not FII level measuring… DO NOT

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Factor XII deficiency is associated with

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liver… dec plasmin. Results in thrombosis.

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Two enzymes related to hyperhomocysteinemia

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cystathionine B synthase. Homocysteinuria (none converted to cysteine.. build up) and MTHFR (methylene tetrahydrofolate reductase) homocysteinemia results in presence of folate depletion. (nothing to reduce).

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Treat hyperhomocysteinemia with

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folate, B6 and B12.

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Effect of elevated homocysteine

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damage to endothelial cells. Premature atherosclerosis. Venous thrombosis. Preg related vascular disorders like fetal loss can occur.

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Effect of Elevated Factor VIII levels

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Not manufactured in liver, but liver disease causes really large numbers of F VIII….>400% leads to increased thrombin and venous thrombosis.

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Effects of Increased PAI-1

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Decreased fibrinolysis à increased and persistent clotting (venous thrombosis)

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Elevated levels of F XI, V and II lead to

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venous thrombosis (above 90th percentile…levels) due to genetics.

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Antiphospholipid syndrome

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activates factor II and X when autoantibodies attack phospholipid, which leads to thrombus formation… thrombosis. Thrombocytopenia and infarction. (antibodies are formed and react only with hexagonal phospholipids… annexin V, Beta 2 glycoprotein 1, antiphosphatidylserine and antiphosphatidyinositol. Common in women.. recurrent fetal loss. Venous thromboembolism. Thrombocytopenia. Neurologic = stroke. Prolonged DRVVT. Prolonged aPTT

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Factor X deficiency is associated with

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liver. Vit k dependent. Normally activates thrombin with factor Va. Deficiency leads to severe bleeding and repeated fetal loss. Prolonged PT & aPTT; Abnormal DRVVT

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Acquired hypercoagulable states occur via

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immobilization ****BOLDED***, malignancy, pregnancy, hormone replacement therapy. Antiphospholipd syndrome, DIC, excessive factor administration