Factor Problems

Question 1

Little spontaneous bleeding, but bleeding after surgery can be heavy, not as debilitating as hemophilia A

Answer 1

Afibrinogenemia

Question 2

Afibrinogenemia Lab Results

Answer 2

PLT count normal
PT and PTT prolonged
Fibrinogen low
TT prolonged

Question 3

Afibrinogenemia Treatment

Answer 3

Cryoprecipitate (contains factor 8 and fibrinogen)

Question 4

alteration of the structure of fibrinogen, mild bleeding tendencies

Answer 4

Dysfibrinogenemia

Question 5

Dysfibrinogenemia Lab Results

Answer 5

PLT Count normal
PT and PTT are prolonged
TT is prolonged
Fibrinogen normal

Question 6

the patient is both over clotting and over fibrinolysing (most common)

Answer 6

Disseminated Intravascular Coagulation

Question 7

Clinical Classification of DIC

Answer 7

Tissue Injury, Malignancy, Obstetrical, Infections

Question 8

Clinical Manifestations of DIC

Answer 8

hemorrhaging from unrelated sites and thrombosis in intravascular of major organs

Question 9

DIC Lab Reuslts

Answer 9

PLT low
BT prolonged
PT and PTT Prolonged
Fibrinogen low
TT prolonged 
D-dimer present

Question 10

Therapy for DIC

Answer 10

Heparin, Cryo, Platelets

Question 11

increased fibrinolysis, urokinase

Answer 11

primary fibrinolysis

Question 12

Primary Fibrinolysis Lab Results

Answer 12

PT and PTT would be prolonged 
Fibrinogen low
TT Prolonged 
FDP Increased
D-dimer-

Question 13

sex-linked spontaneous bleeding into joints and muscles

Answer 13

Hemophilia A

Question 14

0-2% activity with spontaneous bleeding

Answer 14

Severe Hemophilia A

Question 15

2-5% activity with bleeding from trivial injuries

Answer 15

Moderate hemophilia A

Question 16

5-25% activity with bleeding from traumatic injuries

Answer 16

Mild Hemophilia A

Question 17

Hemophilia Lab Reuslts

Answer 17

PTT prolonged

Question 18

Factor Assay

Answer 18

.05 patient plasma and .05 normal plasma in the PTT to show that it is normal
Redo with a known depleted plasma until you see the test is prolonged
Shows the factor that is missing

Question 19

Hemophilia Treatment

Answer 19

Factor VIII concentrates
Cryo - contraindicated
Recombinant factor VII

Question 20

clinicial symptoms indistinguishable from hemophilia A

Answer 20

Hemophilia B - Christmas Disease

Question 21

Hemophilia B Treatment

Answer 21

Proplex T

recombinant VII

Question 22

easy bruising, bleeding from the gums and GI tract, heavy periods - mucosal bleeding

Answer 22

von Willebrand Disease

Question 23

most common type of von Willebrand

Answer 23

VWF and VIII:C levels reduced - 70%

Question 24

von Willebrands Lab Results

Answer 24

PTT prolonged
Ristocetin aggregation
Ristocetin cofactor assay

Question 25

Ristocetin aggregation

Answer 25

PRP with ristocetin - abnormal test = von Willebrand's

Question 26

Ristocetin Co-Factor Assay

Answer 26

PPP with ristocetin and normal platelets to rule out Bernard Soulier's

Question 27

vWF Disease Treatment

Answer 27

Desmopressin (constricts the vessels)

Question 28

Lupuslike Anticoagulants

Answer 28

prolonged PTT not corrected with mixing studies because of reaction against the phospholipids in the reagent

Question 29

Hypercoagulation

Answer 29

associated with the inappropriate formation of thrombi in the vasculature

Question 30

Factors of Hypercoagulation

Answer 30

Age, Immobilization, Elevated estrogens

Question 31

Types of Hypercoagulation

Answer 31

Anti Thrombin Deficiency Protein C Deficiency Factor V Leiden mutation Prothrombin variant

Question 32

Protein C deficiency

Answer 32

normally inhibits factor V and VIII

Question 33

Factor V Leiden

Answer 33

most recently described genetic defects, Protein C cannot inactivate; needs PCR

Question 34

Prothrombin Variant

Answer 34

substitution of A for G at position 20210 to increase prothrombin

Question 35

COX-2

Answer 35

made in the vascular endothelium, smooth muscle and some platelets

Question 36

Plavix

Answer 36

blocks the P2Y12 ADP binding site on the platelet