3S [LEC]: Hemorrhagic/ Bleeding Disorders

Question 1

Bleeding typically in skin or at body orifices

Answer 1

Mucocutaneous bleeding

Question 2

Bleeding that may indicate a disorder of primary hemostasis

Answer 2

Mucocutaneous bleeding

Question 3

Mucocutaneous bleeding may indicate a disorder of ___ hemostasis

Answer 3

Primary

Question 4

Anatomic bleeding may indicate a disorder of ___ hemostasis

Answer 4

Secondary

Question 5

Bleeding disorders that are associated with some disease or physical trauma, not duplicated in relatives

Answer 5

Acquired bleeding disorders

Question 6

Most common acquired bleeding disorder

Answer 6

Trauma-Induced Coagulopathy

Question 7

In Trauma-Induced Coagulopathy, systemic shock leads to acute reduction of ___

Answer 7

ADAMTS13

Question 8

Ratio of RBC to plasma to platelets in trauma-induced coagulopathy

Answer 8

1:1:1 (random donor), 6:6:1 (plateletpheresis)

Question 9

Liver disease affects the synthetic function, which results to what level of coagulation factors?

Answer 9

<40 units/dL

Question 10

Which coagulation factors are affected in liver disease coagulopathy?

Answer 10

II, VII, IX, X, Protein C, Protein S

Question 11

Which coagulation factors are regulated by proteins C and S?

Answer 11

V, VIII

Question 12

The first coagulation factor to exhibit decreased activity in liver disease coagulopathy

Answer 12

VII

Question 13

Distinguishes liver disease from vitamin K deficiency

Answer 13

Factor V assay

Question 14

Fibrinogen levels in end-stage liver disease

Answer 14

<100 mg/dL

Question 15

Nijmegen-Bethesda (FVIII inhibitor) assay is used for which hemorrhagic condition?

Answer 15

Acquired autoantibodies/ inhibitors

Question 16

Most common hereditary hemorrhagic disorder

Answer 16

Von Willebrand Disease

Question 17

Binds through glycoprotein, which is needed for platelets to adhere to collagen in damaged vessel

Answer 17

Von Willebrand factor

Question 18

Inheritance pattern of Von Willebrand Disease

Answer 18

Autosomal Dominant

Question 19

What coagulation factor is carried by the vWF?

Answer 19

Factor VIII

Question 20

Most common type of vWD

Answer 20

Type 1

Question 21

Von Willebrand disease where factor VIII are proportionally reduced

Answer 21

Type 1

Question 22

“Null allele” vWF gene translation or deletion mutations

Answer 22

Type 3

Question 23

Type of von Willebrand disease which renders vWF susceptible to increased proteolysis by ADAMTS13

Answer 23

Type 2A

Question 24

Type of von Willebrand disease where there is a predominance of small molecular weight plasma multimer and less platelet adhesion activity

Answer 24

Type 2A

Question 25

Type of von Willebrand disease where there is a "gain-of-function" mutation

Answer 25

Type 2B

Question 26

Type of von Willebrand disease where there is a raise in the affinity of HMW-VWF for platelet GPIb/IX/V

Answer 26

Type 2B

Question 27

Type of von Willebrand disease where the vWF spontaneously bind resting platelets, rendering them unavailable

Answer 27

Type 2B

Question 28

Type of von Willebrand disease where there is a poor platelet receptor binding

Answer 28

Type 2M

Question 29

Normandy variant of von Willebrand disease

Answer 29

Type 2N

Question 30

"Autosomal hemophilia"

Answer 30

Type 2N von Willebrand disease

Question 31

Von Willebrand disease where the protein's factor VIII binding site function is impaired

Answer 31

Type 2N

Question 32

Von Willebrand disease where there is a factor VIII deficiency despite a normal vWF antigen concentration

Answer 32

Type 2N

Question 33

Von Willebrand disease where there is a normal vWF activity

Answer 33

Type 2N

Question 34

Von Willebrand disease where there is a moderate thrombocytopenia caused by chronic platelet activation

Answer 34

Type 2B

Question 35

Von Willebrand disease where there is mild to moderate systemic bleeding

Answer 35

Type 1

Question 36

Von Willebrand disease where the factor VIII is proportionally diminished or absent

Answer 36

Type 3

Question 37

Platelet-type VWD/ pseudo-VWD is clinically similar disorder with ___ VWD

Answer 37

Type 2B

Question 38

Desmopressin acetate is a drug indicate for moderate bleeding. However, it is contraindicated to which VWD subtype?

Answer 38

2B

Question 38

Second to VWD in prevalence among congenital bleeding disorders

Answer 38

Hemophilia A

Question 39

Which VWD subtype/s cause severe bleeding

Answer 39

Type 3 & 2B

Question 39

How will a patient with factor IX deficiency be managed? i. Desmopressin acetate ii. Human plasma-derived FVIII iii. FIX concentrates

Answer 39

iii

Question 39

Hemophilia most prevalent among Askenazi Jews

Answer 39

Hemophilia C

Question 39

"Rosenthal syndrome"

Answer 39

Hemophilia C

Question 39

How will a patient with factor VIII deficiency be managed in cases of severe bleeding? i. Desmopressin acetate ii. Human plasma-derived FVIII iii. FIX concentrates

Answer 39

ii

Question 40

How will a patient with factor VIII deficiency be managed in cases of mild to moderate bleeding? i. Desmopressin acetate ii. Human plasma-derived FVIII iii. FIX concentrates

Answer 40

i

Question 40

PTT is prolonged in all factor deficiencies except ___

Answer 40

VII, XIII

Question 41

Defined as any single or multiple coagulation factor or platelet deficiency

Answer 41

Coagulopathy

Question 42

Which coagulation factors will decline within 5 days in FFP?

Answer 42

VWF, Factor V, Factor VIII

Question 43

The first coagulation factor to exhibit a decreased activity in liver disease

Answer 43

Factor VII

Question 44

A condition that causes generalized soft tissue bleeding associated with a prolonged TT and an exceptionally prolonged reptilase clotting time

Answer 44

Dysfibrogenemia

Question 45

Factors that may be unaffected or elevated in mild to moderate liver disease

Answer 45

VWF, Factor VIII, Factor XIII

Question 46

A significant complication of liver disease that is caused by decreased liver production of regulatory antithrombin, protein C, and protein S

Answer 46

DIC

Question 47

A hallmark of unregulated coagulation and fibrinolysis, which will be positive in DIC

Answer 47

D-dimer assay

Question 48

An oral anticoagulant that disrupts vitamin K epoxide reductase and vitamin K quinone reductase reactions

Answer 48

Warfarin/ Coumadin

Question 49

Most common acquire autoantibody

Answer 49

Autoanti-factor VIII (acquired hemophilia)

Question 50

Most prevalent mucocutaneous bleeding disorder

Answer 50

Von Willebrand Disease

Question 51

T/F: VWF is the largest molecule in human plasma

Answer 51

True

Question 52

Where is the VWF synthesized and stored?

Answer 52

Synthesized: ER Stored: Cytoplasmic Weibel-Palade bodies

Question 53

If synthesized by megakaryocytes, VWF is stored in the ___

Answer 53

a-granules

Question 54

A VWD type I variant in which the VWF secretion rate is near normal, but its plasma half-life is reduced from the anticipated 8-12 hours to 1-3 hours

Answer 54

Subtype 1C