DISORDERS OF THROMBOSIS , HEMORRHAGIC DISORDERS Flashcards
memorization
Principal inhibitor of thrombin is deficient
Antithrombin-III Deficiency
Proteins that inactivates factor Va and VIIIa
Protein C and S
A mutated form of FV which resists protein C and S inactivation
Factor V Leiden
An abnormal functional fibrinogen molecule is produced and is resistant to fibrinolysis
dysfibrinogenemia
abnormal platelet-vessel wall interaction; leads to clotting disease
Homocystinuria
Associated with thrombosis due to prostacyclin inhibition; prolonged APTT that does not correct with normal plasma substitution
Lupus anticoagulant
Factor XI deficiency (Hemophilia C) is also known as
Rosenthal Syndrome
More than half of the cases of hemophilia C have been described in
Ashkenazi Jews
Also known as Hemophilia A
Factor VIII:C Deficiencyh
What is the treatment for Hemophilia A?
- Cryoprecipitate
- FVIII Concentrate
Factor IX deficiency is also known as
Hemophilia B / Christmas Disease
Treatment for Factor IX deficiency
- FFP
- FIX concentrate
Defects of both Factor VIII:C and vWF
Von Willebrand Disease
Most frequently encountered hereditary coagulopathy
Von Willebrand Disease
Recommended initial workup for the diagnosis of Von Willebrand Disease
CBC, APTT, PT
Abnormal PT
Normal APTT
Normal TCT
Factor VII deficiency
Abnormal APTT
Abnormal PT
Normal TCT
- Factor X (stuart-prower factor) deficiency
- Factor V Deficiency (Owren’s Disease)
- Factor II (prothrombin) deficiency
(All common pathway coag factors except Factor I)
Abnormal APTT
Abnormal PT
Abnormal TCT
Factor I deficiency
Clot dissolves in 5 M urea
Factor XIII Deficiency
In hepatic disease, all coagulation factors except Factor III and VWF are decreased or increased?
Decrease
A coagulation study procedure that is a sensitive test for liver function
PT (Prothrombin Time)
Vitamin K is needed for what process for the PT group?
Gamma carboxylation
Coagulation factors and platelets are destroyed leading to decreased coagulation proteins; consumptive coagulopathy
Disseminated Intravascular Coagulation (DIC)
In Primary fibrinogenolysis, D-dimer is negative/decreased because
There is no clot formation, and fibrinogen is being broken down
In DIC, are all coagulation tests prolonged or shortened?
prolonged
Is D-dimer in DIC positive/increased or decreased/negative?
positive/increased
Fibrinogen in DIC is
A. decreased
B. increased
C. decreased or normal
D. normal
C. decreased or normal
