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Disorders of Hemostatic System
Hematology Test 4
92
Health Care
Undergraduate 4
04/19/2009

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Cards

Term
Patients of what disease are often employed at circuses as "India rubber men"?
Definition
Ehler's-Danlos Syndrome
Term
A connective tissue disorder that causes bleeding due to increased fragility of subcutaneous vessels. The patients joints are hyperextensible.
Definition
Ehler's-Danlos Syndrome
Term
Is Ehler's-Danlos Syndrome acquired or inherited?
Definition
inherited
Term
An inherited disorder characterized by serious bleeding secondary to abnormal elastic fibers in the skin and the media of the arteries.
Definition
Pseudoxanthoma elasticum
Term
A connective tissue disorder with many other physical manifestations. Causes mild bleeding tendencies. Abraham Lincoln may have had this disorder.
Definition
Marfan's syndrome
Term
"devil's pinches"; an acquired disorder of unknown cause; usually causing bruising in females
Definition
Purpura simplex
Term
3 inherited bleeding disorders due to problems of the subendothelium
Definition
Ehler's-Danlos Syndrome, Pseudoxanthoma elasticum, Marfan's syndrome
Term
Bruises on hands and forearms, usually seen in older patients; caused by the loss of dermal and vascular support.
Definition
Senile purpura
Term
Vitamin C deficiency resulting in defective collagen and otehr supportive structures. Patients may have pale skin, loss of teeth, and sunken eyes.
Definition
scurvy
Term
Three acquired bleeding disorders due to problems of the subendothelium
Definition
Purpura simplex, senile purpura, scurvy
Term
Osler-Weber-Rendu syndrome is another name for what inherited disorder?
Definition
Hereditary Hemorrhagic Telangiectasia
Term
An autosomal dominant disorder characterized by cutaneous lesions called A-V malformations, which hold blood and appear as red-colored spots.
Definition
Hereditary Hemorrhagic Telangiectasia
Term
Why is Hereditary Hemorrhagic Telangiectasia more common than most other vascular diseases?
Definition
Because it's autosomal dominant
Term
allergic purpura, hepatitis B- associated vaculitis, various infections and reactions to drugs; all these inflammatory disorders are acquired disorders of what part of the vascular system?
Definition
Endothelial cells
Term
The deficiency of the Glycoprotein IIb/IIIa complex on the platelet surface. Abnormal bleeding time and platelet aggregation tests. Autosomal recessive disease
Definition
Glansman's Thrombasthenia
Term
The deficiency of the Glycoprotein Ib receptor. Autosomal recessive.
Definition
Bernard Soulier Disease
Term
Bernard Soulier patients are clinically identical to patients with what other disease?
Definition
Von Willebrand's Disease
Term
Three diseases that produce substances which coat the platelet receptors?
Definition
uremia, multiple myeloma, anti-platelet antibodies
Term
The deficiency of the von Willebrand's Factor molecule which binds the platelet to the collagen
Definition
Von Willebrand's Disease
Term
Which is more common, Bernard Soulier Disease or Von Willebrand's Disease?
Definition
Von Willebrand's Disease
Term
DDAVP is used to treat what disease?
Definition
von Willebrand's Disease
Term
How is von Willebrand's disease inherited?
Definition
Autosomal dominant
Term
deficiency of Factor VIII due to a defective gene on the X chromosome
Definition
Hemophilia A
Term
Which disease is characterized by bleeding into the joints?
Definition
Hemophilia
Term
Which disease is characterized by bleeding from the gums, nose bleeds, and heavy menstrual bleeding in females?
Definition
Von Willebrand's Disease
Term
epistaxis is bleeding from what body part?
Definition
the nose
Term
A bleeding disease that has a normal bleeding time, normal aggregation studies, prolonged APTT and decreased factor VIII level
Definition
hemophilia A
Term
People with hemophilia B have a decrease in what factor?
Definition
Factor IX
Term
which version of hemophilia is treated by using plasma replacement or commercial Factor IX replacement?
Definition
Hemophilia B
Term
name five factor deficiencies that are autosomal recessive and extremely rare.
Definition
Factor II, Factor V, Factor VII, Factor X, Factor XIII
Term
Hemophilia C is a deficiency of what factor?
Definition
Factor XI
Term
Autosomal dominant factor deficiency with over 40 abnormal variants with a spectrum of clinical expression.
Definition
Factor I (fibrinogen) deficiency
Term
How is Hemophilia C inherited?
Definition
Autosomal dominant
Term
A phenomenon most commonly seen in the elderly and in post partum females; similar to Hemophelia A but much more difficult to treat.
Definition
Factor VIII antibody
Term
An antibody, usually IgG, to the phospholipid platelet membrane. Can prolong both PT and APTT, but does not cause bleeding.
Definition
Lupus anticoagulant
Term
Vitamin K deficiency causes functional deficiency of what four factors?
Definition
Factors II, VII, IX and X
Term
If a woman poisons her husband with rat poison, he will die of what vitamin deficiency..?
Definition
vitamin K deficiency
Term
In what organ are factors I, II, V, VII, IX and X produced?
Definition
in the liver
Term
What does DIC stand for?
Definition
disseminated intravascular coagulation
Term
In primary fibrinolysis, are d-dimers produced?
Definition
no
Term
A very common chronic condition of arterial damage and the formation of atherosclerotic plaque. The acute phase of the blockage is driven by a platelet clot at the injury site which blocks the blood supply.
Definition
Atherosclerosis
Term
an acquired disease of endothelial cells resulting in the loss of prostacyclin function and the release of vWF.
Definition
Thrombotic Thrombocytopenia Purpura (TTP)
Term
oral anticoagulants which inhibit Vitamin K
Definition
Warfarin, Coumadin
Term
an anti-platelet drug that blocks cyclo-oxygenase
Definition
aspirin
Term
an anti-platelet drug that inhibits platelet adhesion
Definition
persantin
Term
what test is used to monitor coumadin or warfarin therapy, and what is the desired result?
Definition
Prothrombin time/ the goal is to get a prolongation of PT of 2x normal
Term
an anticoagulant that binds and activates Antithrombin III, which inhibits the activated serine proteases.
Definition
Heparin
Term
what test is used to monitor patients on heparin therapy, and what is the desired result?
Definition
APTT, the goal is to be prolonged 2x normal
Term
A clot busting drug that was once produced from horse urine but is now produced from human renal cells in tissue culture
Definition
urokinase
Term
name two anticoagulant drugs
Definition
warfarin, heparin
Term
name two anti-platelet drugs
Definition
aspirin and persantin
Term
name three clot busting drugs
Definition
urokinase, streptokinase, tissue plasminogen activator
Term
the latest and most expensive clot busting drug. Made with new recombinant DNA technology
Definition
tissue plasminogen activator
Term
a product of beta hemolytic streptococci; still a widely used clot busting drug
Definition
streptokinase
Term
name two general categories of disorders of the hemostatic system
Definition
bleeding disorders and clotting disorders
Term
another word for clotting
Definition
thrombosis
Term
two subsets of antiphospholipid antibody syndrome
Definition
Lupus anticoagulant and Anti-cardiolipin antibody
Term
an autosomal dominant disorder that can lead to both arterial and venous thrombosis; characterized by spontaneous platelet aggregation in vitro
Definition
sticky platelet syndrome
Term
A mutated form of Factor V that leads to Activated Protein C resistance
Definition
Factor V Leiden
Term
what is the most common inherited form of hypercoagulability?
Definition
Factor V Leiden
Term
what inherited disorder is present in 3-15% of caucasians of northern european origin, but rare in persons of african or asian ancestry?
Definition
Factor V Leiden
Term
The screening test called Activated Protein C Resistance Test is used to test for what disorder?
Definition
Factor V Leiden
Term
A mutated prothrombin gene seen in 2-3% of general population and 5-18% of people with family history of deep vein thrombosis
Definition
prothrombin 20210A gene
Term
anti-clotting drugs that inhibit ADP receptors
Definition
Plavix and Ticlid
Term
How is heparin therapy administered?
Definition
IV or sub-q injection
Term
an overdose of warfarin is counteracted with what?
Definition
Vitamin K
Term
what drug is used to counteract a heparin overdose?
Definition
Protamine sulfate
Term
vasoconstriction and formation of platelet plug are what phase of hemostasis?
Definition
primary
Term
fibrin clot formation and fibrinolysis are in what phase of hemostasis?
Definition
Secondary
Term
two types of platelet function tests
Definition
platelet aggregation and clot retraction
Term
what does PRP stand for, and how do you get it?
Definition
Platelet rich plasma; you get it by centrifuging slowly
Term
what aggregating agents are used in the platelet aggregation test?
Definition
ristocetin, ADP, collagen, and arachidonic acid
Term
In a platelet aggregation test on a patient with von willebrand's disease, which aggregating agent will cause incomplete or no aggregation?
Definition
ristocetin
Term
In a mixing study, if the pooled normal plasma does not correct the result, what is indicated?
Definition
inhibitor
Term
in the mixing study test, if the addition of pooled normal plasma corrects the result, what is indicated?
Definition
factor deficiency
Term
what factor deficiency is associated with umbilical stump bleeding?
Definition
Factor XIII deficiency
Term
what factor deficiency is the 5 M Urea Test used to test for?
Definition
Factor XIII deficiency
Term
what are lupus anticoagulants directed against?
Definition
phospholipids
Term
What two tests are good to use to test for lupus anticoagulant?
Definition
Kaolin clot time and platelet neutralization
Term
Fletcher factor is another name for what?
Definition
prekallekrien
Term
If a prolonged APTT is corrected by incubating the serum for 15 minutes instead of 5, what is indicated?
Definition
fletcher factor deficiency
Term
what does DRVVT stand for, and what is it used for?
Definition
Dilute Russel's Viper Venom Test, used to test for Lupus Anticoagulant
Term
what test can be used to rule out heparin contamination?
Definition
Reptilase time
Term
What is the cofactor to Protein C?
Definition
Protein S
Term
what activates Protein C?
Definition
Thrombin and thrombomodulin
Term
do immunologic assays measure function?
Definition
NO
Term
name four things that could cause acquired antithrombin III deficiency?
Definition
DIC, liver diease, sepsis, trauma
Term
in the antithrombin III chromogenic assay, what is the relationship between the amount of color and the amount of patient antithrombin III?
Definition
Inversely proportional
Term
In the Activated Protein C resistance test, what result would indicate the presence of Factor V Leiden?
Definition
Short time
Term
what is a quantitative but not functional test to check for von willebrand factor antigen?
Definition
laurel rocket technique
Term
How is prothrombin gene mutation detected?
Definition
using PCR
Term
what is a functional assay to test for von willebrand disease?
Definition
ristocetin cofactor
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