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Block 7 Week 4 > Fung: Coagulation II > Flashcards

Fung: Coagulation II Flashcards

1
Q

Clinically, most bleeding disorders can be divided into these two categories…

A

coagulation-type bleeding

platelet-type bleeding

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2
Q

In this type of bleeding disorder, petechiae and mucosal bleeding are rare, but hemarthroses, deep hematomas and delayed bleeding are common; seen in males

A

coagulation type bleeding disorder

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3
Q

In this type of bleeding disorder, petechiae and mucosal bleeding are common, but hemarthroses, deep hematomas and delayed bleeding are RARE; seen in females

A

platelet type bleeding disorder

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4
Q

This is the most common inherited bleeding disorder; it is a defect in PLATELET ADHESION; combined platelet and coagulation defect; 4 main types

A

von Willebrand disease

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5
Q

Where is von Willebrand factor synthesized? Where is it sotred? What does it do?

A

synthesized in endothelial cells and megakaryocytes; stored in Weibel-Palade bodies of endothelial cells and alpha granules of megakaryocytes; mediates platelet adhesion by binding GPIb

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6
Q

Another name for von Willebrand factor

A

Ristocetin cofactor

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7
Q

What does vWF complex with in the circulation to decrease its degradation?

A

factor 8

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8
Q

Most common type and results in a mild bleeding disorder
QUANTITATIVE disorder showing reduced amounts of vWF
Laboratory evaluation will show
Normal PT
Prolonged PTT and BT
Decreased FVIII
Decreased vWF and activity

A

von Willebrand disease: Type 1

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9
Q

Qualitative (functional) defect of vWF
4 subtypes
IIa
IIb
IIM – rare defect that prevents vWF binding to GPIb
IIN – rare defect with reduced vWF binding to FVIII

A

von Willebrand disease: Type 2

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10
Q

What is the main difference between von Willebrand disease type IIa and type IIb?

A

IIa: absence of high molecular weight multimers, ristocetin cofactor (vWF) activity decreased;

IIb: decreased high molecular weight multimers, enhanced ristocetin cofactor, DO NOT give DDAVP

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11
Q

Defect of platelet adhesion
Due to decreased platelet GPIb/V/IX
Patients clinically manifest with thrombocytopenia and giant platelets
Laboratory evaluation
PFT: aggregation on all agonists except ristocetin
Similar picture to vWD on PFT
Peripheral smear will show large platelets (unlike vWD)

A

Bernard Soulier syndrome

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12
Q

In Bernard Soulier syndrome, platelet adhesion is defective due to a decrease in (blank)

A

GPIb/V/IX

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13
Q

How can you differentiate von Willebrand disease from Bernard Soulier syndrome?

A

Bernard Soulier syndrome - giant platelets

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14
Q

Autosomal recessive disorder due to deficient GP IIb/IIIa (fibrinogen receptor)
Platelets lack the PLA1 antigen
Laboratory evaluation
PFT: fail to aggregate with all agonists but ristocetin

A

Glanzmann thrombasthenia

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15
Q

What is deficient in Glanzmann thromasthenia?

A

GP IIb/IIIa (fibrinogen receptor)

**platelets don’t have PLA1 antigen

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16
Q

You can also have defects in granules of platelets. In alpha granule disease, aggregation is blunted with all agents except (blank)

A

ADP

  • *one of the components of dense granules
  • *associated with gray-platelet syndrome
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17
Q

If you have dense granule disease, you will not get a (blank)

A

second wave of aggregation

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18
Q

These two drugs can cause excessive bleeding by inhibiting COX-1 (the enzyme involved in thromboxane A2 production)

A

Aspirin
NSAIDs

**by blocking this enzyme, you will not get dense granule release and the secondary wave of aggregation

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19
Q

Which has a reversible effect, aspririn or NSAIDs?

A

NSAIDs

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20
Q 
What are these?
Immune thrombocytopenic purpura (ITP)
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic uremic syndrome (HUS)
Heparin induced thrombocytopenia (HIT)
A

disorders characterized by thrombocytopenia (low platelets)

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21
Q 
Diagnosis of exclusion: healthy patient with isolated thrombocytopenia with no other obvious cause
Antigenic target of the antibody varies
GP IIb
GP IIIa
GP Ib
GP V
A

immune thrombocytopenic purpura

**when there is no other cause for the thrombocytopenia, consider this

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22
Q 
Syndrome resulting from widespread formation of microvascular platelet thrombi affecting
Central nervous system
Kidneys
Gastrointestinal tract
Other organs
A

thrombotic thrombocytopenic purpura

23
Q

What are some of the symptoms expressed in TTP?

A 
thrombocytopenia
microangiopathic hemolytic anemia
neuro abnormalities
renal abnormalities
fever
24
Q

In thrombotic thrombocytopenic purpura, there is an acute deficiency in vWF-cleaving metalloprotease. What does this lead to?

A

an accumulation of ultra-large vWF multimers which bind platelets and lead to thrombi in the microvasculature and thrombocytopenia

25
Q

Thrombotic microangiopathy characterized by
Thrombocytopenia
Acute renal failure
Microangiopathic hemolytic anemia
Most commonly associated with bloody-diarrhea caused by shiga-toxin producing bacteria (E. coli O157:H7 or S. dysenteriae)

A

Hemolytic uremic syndrome

26
Q

Hemolytic uremic syndrome is most commonly associated with what??

A

shiga toxin producing bacteria (like E. coli O157:H7 or S. dysenteriae)

**causes bloody diarrhea

27
Q

Occurs in 1-3% of patients treated with unfractionated heparin for greater than 5 days

A

heparin induced thrombocytopenia

28
Q

Heparin induced thrombocytopenia is an immune mediated disorder caused by (blank) antibodies against the complex of heparin and PF4

A

IgG

29
Q

Which is more common in heparin induced thrombocytopenia, bleeding or thrombosis?

A

thrombosis

30
Q

3 symptoms of herparin induced thrombocytopenia?

A

thrombocytopenia
thrombosis
allergic reactions

31
Q

Hemophilia A is a congenital X-linked recessive deficiency in (blank), which results in absent circulating (blank) and lifelong bleeding

A

factor 8; factor 8

32
Q

How do patients with hemophilia A present?

A

with delayed bleeding

joint and muscle bleeding

33
Q

What will the following values be like in hemophilia A?

PTT
platelet count
PT
TT

A

increased PTT

normal platelet count, PT and TT

34
Q

Hemophilia B is due to a congenital X-linked recessive deficiency in (blank), which results in decreased or absent (blank) and lifelong bleeding

A

factor 9; factor 9

35
Q

How does hemophilia B present?

A

clinically identical to hemophilia A - delayed bleeding, joint and muscle bleeding

prolonged PTT, normal PT, TT

36
Q

Liver disease can cause this…

A

decreased synthesis of most clotting factors, including fibrinogen

chronic DIC due to impaired clearance of D-dimer

37
Q

Vit K deficiency leads to impaired production of Vit K dependent clotting factors. What are they?

A 
factor II
Factor 7
factor 9
factor 10
protein C
protein S
38
Q

List 4 things that can cause Vit K deficiency

A

hemorrhagic disease of the newborn **newborns get a shot of Vit K to avoid this
antibiotics **knocks out gut flora, so decreased Vit K absorption
malabsorption/malnutrition
warfarin therapy

39
Q

Acquired syndrome characterized by the intravascular activation of coagulation

A

disseminated intravascular coagulation

40
Q

Causes of disseminated intravascular coagulation?

A 
endotoxin causing sepsis
trauma
burns
obstetrical complications
vascular malformations
animal venom
41
Q

In DIC, the patient will have prolonged exposure to (blank) resulting in generalized activation of the coag system and thrombin generation. In addition, there will be no activation of (blank)

A

tissue factor; tissue factor pathway inhibitor

42
Q

What do fibrin formation and fibrinolysis cause in DIC?

A

microthrombi and consumption of clotting factors and platelets

43
Q

Possible clinical manifestations in DIC

A 
hemorrhage
renal/hepatic/respiratory dysfunction
shock
CNS dysfunction
petechiae
purpura
skin necrosis
44
Q

What does the differential diagnosis of thrombophilia depend on?

A

the type of thrombosis (arterial or venous)

45
Q

Inherited autosomal dominant condition responsible for 50% of the cases of hereditary thrombophilia
Due to a point mutation in the FV gene that makes FV Leiden resistant to proteolytic cleavage by APC
Heterozygotes have 5-10 increased risk of thrombosis

A

Activated protein C resistance

46
Q

What does activated protein C (APC) do? In activated protein C resistance (APCR), what is the problem?

A

Activated protein C usu degrades factor 5a and 8a; if these cannot be degraded, may lead to longer duration of thrombin generation and increased risk of thrombosis

47
Q

Autosomal dominant disorder characterized by recurrent venous thrombosis
No inactivation of Factors II, IXa, Xa, XIa, XIIa
Heterozygotes have 5-10 increased risk of thrombosis
Homozygosity is considered incompatible with life

A

anti-thrombin deficiency

48
Q

What is the problem in anti-thrombin deficiency?

A

no inactivation of factors II, 9a, 10a, 11a, and 12a which leads to recurrent venous thrombosis

49
Q 
An autoimmune thrombophilic condition in which patients have circulating antibodies against plasma proteins that bind to phospholipids
Patients have 
Recurrent arterial and venous thrombosis
Pregnancy loss
Immune cytopenias
A

anti-phospholipid syndrome

50
Q

What are some things that you might form antibodies to in antiphospholipid syndrome?

A

Beta-2 glycoprotein

prothrombin

51
Q

What is the difference between primary and secondary antiphospholipid syndrome?

A

primary APL: healthy individuals

secondary APL: associated w disease (lupus, HIV, cancers, drugs)

52
Q

Autosomal dominant condition due to a point mutation in the prothrombin gene
Mutation enhances prothrombin gene transcription leading to elevated levels of prothrombin
Second most common cause of inherited thrombophilia

A

prothrombin variant (G20210A)

53
Q

Inherited autosomal dominant form with heterozygotes with 5-7 fold increased risk of thrombosis
Acquired form may result from
Coumadin therapy
Liver disease
Pregnancy
Deficiency can either be due to qualitative or quantitative defects

A

Protein C/S deficiency

**Protein C inactivates factor 5 and 8, so you lose your checks and balances in the coag cascade and this leads to excessive thrombosis

Block 7 Week 4 (8 decks)

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