DISORDERS OF FIBRINOLYTIC SYSTEM and THROMBOTIC DISORDERS

Question 1

it comprises an inactive proenzyme, plasminogen, which can be converted to the active enzyme, plasmin, which in turn degrades fibrin into soluble fibrin degradation products.

Answer 1

fibrinolytic system

Question 2

excessive amounts of plasminogen activators from damaged cells/malignant cells

Answer 2

primary fibrinolysis

Question 3

secondary fibrinolysis is can be caused by

Answer 3

DIC; uncontrolled, inappropriate formation of fibrin within the blood vessels

example:
infection
neoplasm
snake bite
HTR

Question 4

the disorder of fibrinolytic system can impair the breakdown of fibrin resulting in a build-up of fibrin thus resulting to __

Answer 4

thrombosis

Question 5

Plasminogen Deficiency

what are its subclassification

Answer 5

plasminogen deficiency type 1
plasminogen deficiency type II

Question 6

subclassification of plasminogen deficiency which is the True plasminogen deficiency (hypoplasminogenemia)

Answer 6

plasminogen deficiency type 1

Question 7

subclassification of plasminogen deficiency wherein there’s Proportionate decrease in both level of plasminogen and its activity

Answer 7

plasminogen deficiency type I

Question 8

cause of plasminogen deficiency type I

Answer 8

congenital plasminogen deficiency is caused by MUTATIONS in the PLG gene

Question 9

this gene provides instructions for making a protein called plasminogen

Answer 9

PLG gene

Question 10

dx for plasminogen deficiency 1

Answer 10

genetic testing
research studies

Question 11

other name for plasminogen deficiency type I

Answer 11

hypoplasminogenemia
plasminogen deficiency, type I

Question 12

subclassification of plasminogen deficiency wherein the level of immunoreactive plg is normal whereas the specific functional activity is markedly reduced because of abnormalities in the variant plg molecule

Answer 12

plasminogen deficiency type II

Question 13

refers to an increased tendency to develop thrombi or emboli

Answer 13

thrombosis

Question 14

thrombosis is sometimes called as ____

Answer 14

hypercoagulable state

Question 15

thrombosis is a multifaceted disorder resulting from abnormalities in blood flow, such as

Answer 15

stasis
abnormalities in the coagulation system
plt function
leukocyte activation molecules
blood vessel wall

Question 16

predisposition to thrombosis

Answer 16

thrombophilia

Question 17

abnormality of blood coagulation that increases the risk of thrombosis

Answer 17

thrombophilia

Question 18

thrombophilia, it may be due to ____

Answer 18

physical, biological, and chemical events

Question 19

thrombophilia, it may be due to physical, biological, and chemical events such as :

Answer 19

acute or chronic inflammation
inappropriate or uncontrolled plt activation
uncontrolled triggering of the plasma coagulation system
inadequate control of coagulation-impaired fibrinolysis

Question 20

classification of thrombosis

Answer 20

venus and arterial thrombosis

Question 21

what are the venous thrombosis

Answer 21

deep vein thrombosis
portal vein thrombosis
renal vein thrombosis
jugular vein thrombosis
budd chiari syndrome
paget-schroetter disease
cerebral venous sinus thrombosis

Question 22

formation of blood clot within a deep bein

Answer 22

deep vein thrombosis

Question 23

affects the superficial leg veins from the iliac, poplietal, and femoral veins of the upper leg and calves

Answer 23

deep vein thrombosis

Question 24

signs of deep vein thrombosis

Answer 24

swelling, pain, and redness in the affected area especially the entire legs

Question 25

form of venous thrombosis affecting the hepatic portal vein which can lead to portal hypertension and reduction of supply to the liber

Answer 25

portal vein thrombosis

Question 26

obstruction of the renal vein by a thrombus

Answer 26

renal vein thrombosis

Question 27

jugular vein thrombosis may occur during __

Answer 27

infection, malignancy, or infusion of the intravenous drug`

Question 28

conditions associated with jugular vein thrombosis

Answer 28

systemic sepsis, pulmonary embolism, papilledema

Question 29

this is the form of thrombosis present abdominal pain, hepatomegaly and ascites

Answer 29

budd-chiari syndrome

Question 30

there is a blockage of the inferior vena cava by a thrombus

Answer 30

budd-chiari sundrome

Question 31

conditions that usually occurs after exercise

Answer 31

paget schroetter disease

Question 32

there is an obstruction of the upper extremity vein by a thrombus

Answer 32

paget schroetter disease

Question 33

rare form of thrombosis which result from a blockage of the diural venous sinuses by a thrombus

Answer 33

cerebral venous sinus thrombosis

Question 34

cerebral venous sinus thrombosis has the same symptoms of ___

Answer 34

stroke, headache, and abnormal vision

Question 35

it is the formation of accumulation of thrombi within an artery known as

Answer 35

atherosclerotic plaque

Question 36

arterial thrombosis is also known as

Answer 36

atherothrombosis

Question 37

it causes infarction to different organs in the body

Answer 37

arterial thrombosis

Question 38

arterial thrombosis is common to _____% patient not receiving anticoagulant therapy ex, MI or stroke

Answer 38

5%

Question 39

suspected when thrombotic events occur in young adults and their unusual sites (mesentery, renal, or axillary veins )

Answer 39

genetic or congenital thrombosis

Question 40

it involved the alteration of guanine to adenine at the base of the 20210 of the 3` untranslated region of prothrombin gene

Answer 40

prothrombin 20210 mutation

Question 41

prothrombin 20210 mutation is found in __ % with clotting disorder

Answer 41

10%

Question 42

second most common inherited thrombophilic tendency in patients with family history of DVT

Answer 42

prothrombin 20210 mutation

Question 43

associated with higher levels of prothrombin, averaging to 130%

Answer 43

prothrombin 20210 mutation

Question 44

prothrombin 20210 mutation is linked to other clotting events such as

Answer 44

coronary artery disease venous clots etc

Question 45

hyperhomocysteinuria deficient in 3 enzymes

Answer 45

methionine synthase cystathionine-B-synthase 5, 10 methyltetrahydrofolate reductase

Question 46

increased level of homocysteine

Answer 46

hyperhomocysteinemia

Question 47

may predispose to arterial thrombosis and venous thromboembolism

Answer 47

hypercysteinuria

Question 48

May be due to deficiency in enzymes that will hydrolyze homocysteine in several end products

Answer 48

Hyperhomocysteinuria

Question 49

Plasma homocysteine levels are ≥10 fold in homozygous cystathionine-ß-synthase deficiency. true or false

Answer 49

true

Question 50

inherited cause of hyperhomocysteinuria

Answer 50

low levels of an enzymes necessary for the conversion of homocysteine to cysteine which increase the risk of a clotting event.

Question 51

acquired event of hyperhomocysteinuria

Answer 51

advance age and diet

Question 52

factor V leiden mutation is also called as

Answer 52

activated protein C resistance

Question 53

factor V Leiden Mutation or Activated Protein C Resistance is identified on what year

Answer 53

1993

Question 54

factor V Leiden Mutation or Activated Protein C Resistance it was identified in 1993 as the tope leading cause of

Answer 54

blood blots among white population

Question 55

The most common genetic risk of factor for blood clots.

Answer 55

Factor V Leiden Mutation or Activated Protein C Resistance

Question 56

The mutation happens in Factor V protein, which is known as the

Answer 56

Leiden protein

Question 57

Factor V Leiden Mutation or Activated Protein C Resistance The mutated plasma protein is resistant to regulation by _____that is why clotting is uncontrolled

Answer 57

activated protein C

Question 58

posses only the Leiden protein and an 80-fold increased risk of clotting qdisorder compared to the general unaffected population

Answer 58

Factor V Leiden homozygotes

Question 59

are believed to produced about 50% of Leiden protein and have 5-7 fold increased risk of clotting disorder compared to the general population

Answer 59

Factor V Leiden heterozygotes

Question 60

OTHER FACTOR V MUTATION:

Answer 60

Factor V Cambridge mutation Factor V Hongkong mutation

Question 61

Elevated levels of other clotting factors as a prociagulant can also be associated with increased risk of thrombosis list the mentioned clotting factors

Answer 61

* Factor VIII * Factor IX * Factor XI * Factor I * vWF

Question 62

A serine protease inhibitor (serpin) that neutralizes thrombin, Factor Ixa, Xa, Xia & XIIa.

Answer 62

ANTITHROMBIN

Question 63

ANTITHROMBIN Its activity is enhanced by

Answer 63

unfractionated heparin, LMW heparin, and systemic entasaccharide

Question 64

First of the plasma coagulation control protein to be identified and first assayed in the laboratory

Answer 64

ANTITHROMBIN

Question 65

Acquired antithrombin deficiency

Answer 65

* Liver disease * Nephrotic syndrome * Prolonged heparin therapy * Oral contraceptives * DIC (AT is rapidly consumed)

Question 66

Congenital antithrombin deficiency

Answer 66

Defect in heparin binding sites causing severe clotting tendencies that usually presents in early stage of life

Question 67

2 types of antithrombin deficiency

Answer 67

type I and II AT deficiency

Question 68

this type of AT deficiency refers to the genetic alteration usually leads to drastic decrease levels in AT

Answer 68

Type I AT deficiency

Question 69

this type of AT deficiency refers to the genetic alteration leads to abnormal function of the plasma protein.

Answer 69

type II AT deficiency

Question 70

type of protein C deficiency wherein there's low protein C levels and activity.

Answer 70

Type I Protein C deficiency

Question 71

type of protein C deficiency wherein there's ow protein C activity due to genetic alteration of protein C sequence resulting to abnormal functioning.

Answer 71

Type II Protein C deficiency

Question 72

this protein C deficiency appears in newborn infants.

Answer 72

Homozygous Protein C Deficiency

Question 73

Homozygous Protein C Deficiency can cause what type of purpura

Answer 73

purpura fulminans in children

Question 74

caused by rapid drop in protein C and factor VII resulting to temporary super clotting state in the extremities

Answer 74

warfarin induced skin necrosis

Question 75

can also cause neonatal purpura fulminans which is clinically indistinguishable from Protein C deficiency.

Answer 75

protein S deficiency

Question 76

a type of protein S deficiency wherein there's a proportional decrease in the level and activity of Protein S

Answer 76

type I protein S deficiency

Question 77

a type of protein S deficiency wherein the levels of the free and bound forms are normal, but there is an abnormal function due to genetic alteration in the gene sequence

Answer 77

type II protein S deficiency

Question 78

a type of protein S deficiency wherein there's a normal level to total protein S but with clinically significant decrease in free protein S levels

Answer 78

type III protein S deficiency

Question 79

Deficiency leads to increased thombi formation due to low/deficient activity to inhibit thrombin

Answer 79

Heparin Cofactor II Deficiency

Question 80

type of heparin cofactor II deficiency where in there's a decreased in both levels and functional capacity

Answer 80

type I (quantitative)

Question 81

type of heparin cofactor II deficiency where in there's a decreased in functional capacity but with normal levels of heparin cofactor II.

Answer 81

type II (qualitative)

Question 82

Small thrombomodulin fragments circulates in soluble form in plasma of healthy individuals.

Answer 82

thrombomodulin Deficiency

Question 83

thrombomodulin Deficiency increased levels are seen in patients with ___

Answer 83

venous and arterial clotting conditions and DIC