11/13 - Hematopoetic and Lymphoid System

Question 1

what are examples of derangement in clotting mechanisms

Answer 1

1. hemophilia
2. disseminated intravascular coagulation
3. excessive fibrinolysis

Question 2

Inherited or acquired deficiencies of virtually every coagulation factor have been reported as causes of ___

Answer 2

bleeding diatheses

Question 3

Bleeding due to coagulation factor deficiencies manifest as what

Answer 3

◦ Large posttraumatic ecchymoses
◦ Hematomas
◦ Prolonged bleeding from laceration
◦ After a surgical procedures

Question 4

bleeding often occurs where in hemorrhagic diatheses

Answer 4

Bleeding often occurs into the GI and urinary
tract and into weight bearing joints

Question 5

in terms of hemorrhagic diatheses, so hereditary deficiencies affect a SINGLE or MULTIPLE clotting factor

Answer 5

SINGLE

Question 6

what are the inherited deficiencies that cause hemorrhagic diatheses

Answer 6

1. Hemophilia A - Factor VIII
2. Hemophilia B - Factor IX
3. Von Willebrand factor (both coag and platelet function affected)

Question 7

in terms of hemorrhagic diatheses, do acquired deficiencies involve SINGLE or MULTIPLE coagulation factors

Answer 7

MULTIPLE

Question 8

what are acquired deficiencies that can cause hemorrhagic diatheses

Answer 8

◦ Vit K deficiency impairs the synthesis of factors II, VII, IX, X and protein C
◦ Liver disease affects many factors synthesized in liver

Question 9

hemophilia A also called what

Answer 9

factor VIII deficiency

Question 10

what is the most common hereditary disease
associated with life-threatening bleeding, is
caused by mutations in factor VIII, an
essential cofactor for factor IX in the
coagulation cascade

Answer 10

hemophilia A

Question 11

what is trait of hemophilia A? who does it affect?

Answer 11

Inherited as an X linked recessive trait,
hence mainly affects males and homozygous
females

Question 12

T/F: in 30% of cases of hemophilia A, it is caused by NEW mutation and there is no family history

Answer 12

TRUE

Question 13

in hemophilia A, patients have [prolonged/normal] PTT and [prolonged/normal] PT

Answer 13

prolonged; normal

Question 14

Factor VIII deficiency clinical features

Answer 14

Question 15

what is called the Royal Disease since it appeared in one of Queen Victoria’s sons and was propagated in her descendants

Answer 15

VIII deficiency/hemophilia A

Question 16

what is hemophilia B also called

Answer 16

Christmas disease (named after person not holiday) and Factor IX deficiency

Question 17

what disease:

Severe factor IX deficiency produces a
disorder clinically indistinguishable from
factor VIII deficiency

Answer 17

hemophilia B

Question 18

what is the trait of hemophilia B

Answer 18

inherited X linked recessive

Question 19

does hemophilia B show variable clinical severity

Answer 19

YES

Question 20

is PTT in hemophilia B NORMAL or PROLONGED

Answer 20

prolonged

Question 21

how to tx hemophilia B

Answer 21

infusion of recombinant factor IX

Question 22

A bleeding disorder caused by the qualitative
or quantitative deficiency of the von
Willebrand factor

Answer 22

Von Willebrand Disease

Question 23

Von Willebrand factor necessary for proper ___

Answer 23

platelet adhesion to damaged blood vessels

Question 24

what is a carrier for Factor VIII and protects it from degradation

Answer 24

Von Willebrand factor

Question 25

affected ppl with von willebrand disease sypmtoms

Answer 25

◦ Excessive bruising ◦ Prolonged bleeding from mucosal surfaces ◦ Prolonged bleeding after minor trauma

Question 26

what type of vWF is associated with QUANTITATIVE defects

Answer 26

Type 1 and 3

Question 27

what type of vWF is characerized by QUALITATIVE DEFECTS

Answer 27

Type 2

Question 28

do ppl with vWF have mild, moderate or severe symptoms

Answer 28

MILD

Question 29

symptoms of vWD? is this commonly diagnosed?

Answer 29

often underdiagnosed! ◦ Women may have heavy menstrual periods ◦ Epistaxis ◦ Excessive bleeding from wounds

Question 30

who gets vWD? is this AD or AR?

Answer 30

***AD or AR***; both men and women can have it in about equal numbers

Question 31

what is an acute, subacute, or chronic thrombohemorrhagic disorder characterized by the excessive activation of coagulation and the formation of thrombi in the microvasculature

Answer 31

disseminated intravascular coagulation

Question 32

what is disseminated intravascular coagulation also called

Answer 32

DIC or consumptive coagulopathy

Question 33

because of thrombotic diathesis, what happens in DIC

Answer 33

because of thrombotic diathesis, there is consumption of platelets, fibrin, and coagulation factors, and secondarily activation of fibrinolysis

Question 34

DIC signs and symptoms

Answer 34

◦ Tissue hypoxia and infarction caused by microthrombi ◦ Hemorrhage due to depletion of factors required for hemostasis and activation of fibrinolytic mechanism or both

Question 35

common triggers of DIC

Answer 35

◦ Sepsis ◦ Major trauma ◦ Certain cancers ◦ Obstetric complications

Question 36

tx for DIC

Answer 36

anticoagulants