Hemostasis Disorders Past Paper

77 2. Acquired Disorders - a. Inhibitors - usually IgG antibody...

77 2. Acquired Disorders - a. Inhibitors - usually IgG antibody directed against a specific factor or phospholipids (PL) Implications ofa Negative ❖ Lupus anticoagulant (LA) D-Dimer Test to rule out ~ Directed against thrombosis phospholipids ~ Seen in Lupus Erythematosis Hemostasis Disorders (about 5-10%) hut also seen in mahgnancies, infections, l. Inherited Disorders drug therapy and other a. Hemophilia A autoimmune disorders ♦:♦ Deficiency of Factor VIII ~ Screening tests use low ❖ Sex linked recessive - almost concentration phospholipid exclusive to men reagents ( e.g. DRVV1) ♦:♦ Spontaneous bleeding into joints ~ Use platelet neutralization : Treat with commercial Factor techniques to confirm VIII presence (PL n eutra.lizes b. Hemophilia B antibody and test will ♦:♦ Deficiency of Factor IX correct) ♦:♦ Sex linked recessive - almost ~ Can prolong the APTT exclusive to men ~ Predisposes patient to : Clinically identical in clotting inheritance and symptoms to ❖ Factor ·v IJl inhibitor - most Hemophilia A common specific inhibitor, hut ❖ Treat with.Factor IX others have been demonstrated; concentrates APTT mixing studies c. Hemophilia C differentiate factor defi.ciency : Deficiency o[ Factor XI from inhibitor ♦:♦ Incomplete autosomal recessive ~ If corrected by normal ❖ Wide range in clinical severity plasma, a factor deficiency is ♦:♦ High incidence in Ashkenazi indicated Jews ~ If NOT corrected, an ♦:♦ Only contact factor associated inhibitor should be with bleeding investigated d. von Willebrand Disease b. Vitamin K deficiency ♦:♦ Primary defect in the von ❖ Functional deficiency of Factors Willehrand Factor ( v WF) II, VII, IX and X ♦:♦ v WF binds platelets via the ❖ PH'KA molecules (proteins in Glycoprotein lB/VIIX receptor vitamin K absence) - present but ♦:♦ Usually find secondary not functional deficiency of Factor VIII ❖ Vitamin K originates from diet ♦:♦ Autosomal dominant - males and and bacteria in gut females are affected equally ❖ Deficiency seen in poor diet and ♦:♦ Platelet adhesion defect with with broad spectrum antibiotic abnormal PFA test use ♦:♦ Treat with cryoprecipitate or ❖ Same result observed in DDAVP (drug); Factor VIII warfarin therapy concentrates do NOT contain vWF e. Factor XIII deficiency ♦:♦ Autosomal recessive ♦:♦ NOT detected by common coagulation tests ♦:♦ Results in a sign.ificant bleeding Vitmin K factors and disorder ; poor wound healing deficient states ! Detected with the 5M urea test