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123 Cards in this Set

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Factors preventing thrombus formation in small blood vessels.
Heparin-like molecules
Prostaglandin (PG) I2
Proteins C and S
Tissue plasminogen activator (tPA)
Heparin-like molecules enhance ____ activity
enhance ATIII activity
ATIII - function?
neutralizes activated serine protease coagulation factors
PGI2 - two functions?
1. vasodilator
2. inhibits platelet aggregation
Proteins C and S -- Inactivate factors ____ and ____, enhance ____.
inactivate factors V and VII, enhance fibrinolysis
tPA: activates ____ to release ____.
activates plasminogen to release plasmin
Factors enhancing thrombus formation in small vessel injury.
Thromboxane A2
Von Willebrand factor
Tissue thromboplastin (factor III)
Extrinsic and intrinsic coagulation systems
tPA: activates ____ to release ____.
activates plasminogen to release plasmin
TXA2 - two functions
vasoconstrictor, enhances platelet aggregation
vWF = ____ ____ molecule
platelet adhesion molecule
Factors enhancing thrombus formation in small vessel injury.
Thromboxane A2
Von Willebrand factor
Tissue thromboplastin (factor III)
Extrinsic and intrinsic coagulation systems
vWF - where is it synthesized?
Weibel-Palade bodies in endothelial cells
TXA2 - two functions
vasoconstrictor, enhances platelet aggregation
vWF = ____ ____ molecule
platelet adhesion molecule
Factor VIII:c - where is it synthesized?
synthesized in liver + reticuloendothelial cells
Decrease in vWF causes ____ in VIII:c.
decrease
vWF - where is it synthesized?
Weibel-Palade bodies in endothelial cells
vWF - two functions
1. platelet adhesion
2. prevents degradation of VIII:c in plasma
Factor VIII:c - where is it synthesized?
synthesized in liver + reticuloendothelial cells
Decrease in vWF causes ____ in VIII:c.
decrease
Tissue thromboplastin: activates factor ____ in the ____ coagulation system
factor VII in the extrinsic coagulation system
vWF - two functions
1. platelet adhesion
2. prevents degradation of VIII:c in plasma
Platelet receptor GpIb binds to ____.
vWF
Tissue thromboplastin: activates factor ____ in the ____ coagulation system
factor VII in the extrinsic coagulation system
Platelet receptor GpIb binds to ____.
vWF
Platelet receptor GpIIb-IIIa binds to ____.
fibrinogen
Ticlopidine, clopidogrel and abciximab interfere with ____ receptor function.
GpIIb-IIIa
ADO, vWF and fibrinogen are important platelet ____ proteins.
platelet storage protiens
Platelet function: stabilizes intercellular ____ junctions in ____ endothelial cells.
stabilizes intercellular adherens junctions in venular endothelial cells.
Extrinsic system: factor ____ activates factors ____ and ____.
Extrinsic system: factor VII activates factors IX and X.
Intrinsic system factors?
XII, XI, IX, VIII
Factor XIIa activates the ____ system.
kininogen system
Final common pathway factors?
X, V, II, I
Factor ____ crosslinks insoluble fibrin monomers.
Factor XIII
Vitimin K-dependent factors?
procoagulants - II, VII, IX, X

anticoagulants - proteins C and S
Vitamin K: liver-activated by ____ ____.
epoxide reductase
Calcium binds ____ vitamin K-dependent factors.
gamma-carboxylated vit K-dependent factors
Factors consumed in a clot to produce serum?
I, II, V, VIII
Plasminogen activators (3)?
tPA, streptokinase, urokinase
Aminocaproic acid inhibits ____.
plasminogen
d-Dimers are crosslinked ____ monomers.
fibrin
Platelet sequence in hemostasis?
1. adhesion
2. release reaction
3. TXA2 synthesis
4. temporary plug
TXA2 enhances ____ attachment to ____ receptors
enhances fibrinogen attachment to GpIIb-IIIa receptors
Temporary platelet plug is held together by ____.
fibrinogen
Stable platelet plug is held together by ____.
fibrin
Platelet adhesion to vWF - defective in which two disorders?
vWD and Bernard-Soulier disease
Platelet synthesis of TXA2 inhibited by ____.
aspirin
Temporary platelet plug defective in ____ disease
Glanzmann's disease
Formation of stable platelet plug defective in ____ deficiencies.
factor
Aspirin or NSAIDS, B-S syndrome, Glanzmann's disease, renal failure, scurvy, thrombocytopenia and vWD are all causes of prolonged ____ ____.
prolonged bleeding time
Pts taking ASA or NSAIDs will have ____ platelet count.
normal platelet count.
Glanzmann's disease is a ____ ____ disease (inheritence) characterized by absent ____ fibrinogen receptors and absent thrombosthenin.
Autosomal recessive, absent GpIIb-IIIa fibrinogen receptors.
Bernard-Soulier syndrome is an autosomal recessive disease characterized by absent ____ platelet receptors for vWF.
GpIb platelet receptors for vWF
Prolonged bleeding time secondary to renal failure can be reversed with dialysis and ____ ____.
desmopressin acetate
Scurvy may cause ____ and ____.
ecchymoses and hemarthroses
Thrombocytopenia -- increased bleeding time when platelet count is < ____ cells/mm^3.
<90,000 cells/mm^3
Von Willebrand disease is an autosomal dominant disorder characterized by absent or defective ____ and decreased ____.
absent or decreased vWF, decreased VIII:c
vWD is a combined ____ and ____ ____ disorder
combined platelet and coagulation factor disorder
BT is a test of platelet function to formation of ____ ____.
temporary plug
Ristocetin cofactor assay is a test of ____ function.
vWF
PT evaluates which factors?
VII, X, V, II and I

(extrinsic system)
INR standardizes PT for ____ therapy.
warfarin
PTT evaluates which factors?
XII, XI, IX, VIII, X, V, II, I

(intrinsic system)
Which coagulation test is better in monitoring warfarin therapy?
PT
Which coagulation test is better in monitoring heparin therapy?
PTT
FDPs are increased with lysis of ____ and ____ in fibrin thrombi
fibrinogen and fibrin

**this test isn't sensitive.
D-Dimer assay detects cross-links and is specific for lysis of ____ ____.
fibrin thrombi
senile purpura is caused by what?
vessel instability -- normal finding in elderly patients
Chronic iron deficiency, malignancy and splenctomy are common causes of secondary (reactive) ____.
thrombocytosis
What is the MCC of qualitative platelet dysfunction?
ASA
Petechiae is only seen with ____.
thrombocytopenia
Bleeding from superficial scratches and easy bruising are signs of ____ ____.
platelet dysfunction (ASA)
Petechia, bleeding from superficial scratches and easy bruising are signs of ____.
thrombocytopenia
Three causes of coagulation disorders?
1. decreased production
2. inhibition
3. consumption
Late rebleeding and hemarthroses are common signs of ____ ____.
coagulation disorders
What is the inheritance pattern of hemophilia A?
x-linked recessive
In pts with Hemophilia A, the % of ____ never changes.

**correlates to severity of disease
% VIII:c
Hemophilia B is an XR disorder involving factor ____ deficiency.
factor IX deficiency
In Hemophilia A, pts have ____VIII:c and ____PTT.
decreased VIII:c, increased PTT
vWD has ____ inheritance.
AD
MVP, Marfan syndrome and angiodysplasia are associated with which coagulation disorder?
vWD
What is the MC hereditary coagulation disorder?
vWD
vWD is a combined ____ and ____ ____ disorder
combined platelet and coagulation factor disorder
Pts with vWD have ___PTT and ____BT.
increased PTT, increased BT

**also, decreased vWF and VIII:c
Rx for vWD?
desmopressin acetate, oral contraceptive pills (estrogen)
With circulating anticoagulant, PT and/or PTT (are/are not) corrected with mixing study.
ARE NOT

** indicates immune destruction
What are the vitamin K-dependent factors?
II, VII, IX, X, protein C and S

**gamma-carboxylation activates them.
Why can't newborns synthesize vitamin K?
lack bacterial colonization in bowel
What is the MCC of vitamin K deficiency in a hospitalized pt?
broad spectrum antibiotics
Rat poison contains ____.
warfarin
Warfarin inhibits ____ ____, rendering Vitamin K nonfunctional.
epoxide reductase
In pts with cirrhosis, ____ synthesis of vitamin K-dependent factors, ____ activation of vitamin-K.
decreased synthesis of vitamin K-dependent factors, decreased activation of vitamin K
MCC DIC?
1. sepsis

2. malignancy
3. crush injury
DIC: ____ of coagulation factors
consumption
DIC is a ____ disorder.
thrombohemorrhagic
DIC lab findings?
inc PT, PTT, D-dimers, BT

dec platelets
Most sensitive screen for DIC?
D-dimers
DIC Rx
Treat underlying causes!!!

component replacement until underlying cause resolved
Open heart surgery, prostatectomy and diffuse liver disease are common causes of ____ ____.
primary fibrinolysis
Primary fibrinolysis lab findings?
+FDPs, -D-dimers,

normal platelet count

inc PT, PTT
Secondary fibrinolysis lab findings?
+FDPs, +D-dimers

dec platelet count

inc PT, PTT
Secondary fibrinolysis is a compensatory reaction in the presence of ____.
DIC
MC hereditary thrombosis syndrome?
Factor V Leiden

**mutant form of factor V cannot be degraded by protein C and S
Hemorrhagic skin necrosis is associated with ____ therapy in protein C deficiency.
warfarin
Clinical sign of ATIII deficiency -- ____ PTT after standard dose of heparin.
normal PTT -- require greater dose of heparin to activate whatever ATIII is present
MCC thrombocytopenia in children 2-6 yo.
Acute idiopathic thrombocytopenic purpura (ITP)
MCC thrombocytopenia in adults.
Chronic idiopathic thrombocytopenic purpura (ITP)
Newborns infants of mothers with ITP may have transient thrombocytopenia due to transplacental passage of ____ antibodies.
IgG
Neonatal alloimmune thrombocytopenia is caused by feto-maternal incompatibility for ____ ____ ____.
platelet specific antigens.
Post-transfusion purpura primarily occurs in ____ women.
multiparous
MCC thrombocytopenia in hospitalized pts.
Heparin-induced thrombocytopenia

**occurs 5-14 days after Rx
Thrombotic thrombocytopenic purpura (TTP) is an acquired or genetic deficiency in vWF-cleaving metalloprotease in endothelial cells that occurs in ____ ____.
adult females
TTP - clinical pentad
1. fever
2. thrombocytopenia
3. renal failure
4. microangiopathic hemolytic anemia with shistocytes
5. CNS deficits
MCC renal failure in kids.
Hemolytic uremic syndrome (HUS)
MCC of HUS is endothelial damage at areteriole-capillary junctions due to Shiga-like toxin of which bacteria?
E. coli (O157:H7 serotype)

**undercooked beef
HUS triad
1. thrombocytopenia
2. acute renal failure
3. microangiopathic hemolytic anemia
Thrombocytopenia ITP, TTP, HUS

lab findings
decreased platelet count, increased bleeding time
vWD - lab findings
inc BT, PTT
Hemophilia A - lab findings
inc PTT
DIC - lab findings
dec platelet count

inc BT, PT, PTT
Primary fibrinolysis - lab findings
inc BT, PT, PTT
ASA or NSAIDS - lab findings
Inc BT
Warfarin or Heparin - lab findings
inc PT, PTT