11/13 - Hematopoetic and Lymphoid System Flashcards

1
Q

what are examples of derangement in clotting mechanisms

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

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

A

bleeding diatheses

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3
Q

Bleeding due to coagulation factor deficiencies manifest as what

A

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

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4
Q

bleeding often occurs where in hemorrhagic diatheses

A

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

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5
Q

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

A

SINGLE

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6
Q

what are the inherited deficiencies that cause hemorrhagic diatheses

A
  1. Hemophilia A - Factor VIII
  2. Hemophilia B - Factor IX
  3. Von Willebrand factor (both coag and platelet function affected)
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7
Q

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

A

MULTIPLE

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8
Q

what are acquired deficiencies that can cause hemorrhagic diatheses

A

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

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9
Q

hemophilia A also called what

A

factor VIII deficiency

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10
Q

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

A

hemophilia A

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11
Q

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

A

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

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12
Q

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

A

TRUE

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13
Q

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

A

prolonged; normal

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14
Q

Factor VIII deficiency clinical features

A
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15
Q

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

A

VIII deficiency/hemophilia A

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16
Q

what is hemophilia B also called

A

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

17
Q

what disease:

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

A

hemophilia B

18
Q

what is the trait of hemophilia B

A

inherited X linked recessive

19
Q

does hemophilia B show variable clinical severity

A

YES

20
Q

is PTT in hemophilia B NORMAL or PROLONGED

A

prolonged

21
Q

how to tx hemophilia B

A

infusion of recombinant factor IX

22
Q

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

A

Von Willebrand Disease

23
Q

Von Willebrand factor necessary for proper ___

A

platelet adhesion to damaged blood vessels

24
Q

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

A

Von Willebrand factor

25
Q

affected ppl with von willebrand disease sypmtoms

A

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

26
Q

what type of vWF is associated with QUANTITATIVE defects

A

Type 1 and 3

27
Q

what type of vWF is characerized by QUALITATIVE DEFECTS

A

Type 2

28
Q

do ppl with vWF have mild, moderate or severe symptoms

A

MILD

29
Q

symptoms of vWD? is this commonly diagnosed?

A

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

30
Q

who gets vWD? is this AD or AR?

A

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

31
Q

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

A

disseminated intravascular coagulation

32
Q

what is disseminated intravascular coagulation also called

A

DIC or consumptive coagulopathy

33
Q

because of thrombotic diathesis, what happens in DIC

A

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

34
Q

DIC signs and symptoms

A

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

35
Q

common triggers of DIC

A

◦ Sepsis
◦ Major trauma
◦ Certain cancers
◦ Obstetric complications

36
Q

tx for DIC

A

anticoagulants