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[Board Exam] Pathology > [3] Hemostasis and Bleeding Disorders > Flashcards

[3] Hemostasis and Bleeding Disorders Flashcards

1
Q

What is the goal of Primary Hemostasis?

A

Formation of a platelet plug

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2
Q

What is the goal of Secondary Hemostasis?

A

Stabilization of the platelet plug

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3
Q

What is the first reaction of a vessel to injury?

A

Vasoconstriction

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4
Q

What mediates the initial vasoconstriction of the damaged vessel?

A

Release of endothelin from damaged vessels

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5
Q

Platelets bind to VWF using?

A

GP1b Receptor

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6
Q

Where does Von Willebrand Factor come from?

A

Platelets

Weibel-Palade Bodies of Endothelial Cells (Vast majority)

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7
Q

Function of ADP

A

Promotes exposure of GpIIb/IIIa receptor on platelets

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8
Q

Function of GPIIb/IIIa

A

Allows platelet aggregation

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9
Q

Platelet degranulation releases these two mediators that are important for platelet aggregation

A

ADP

TXA2

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10
Q

Function of TXA2

A

Platelet aggregation

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11
Q

What is the linking molecule of GpIIb/IIIa for platelet aggregation?

A

Fibrinogen

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12
Q

Disorders of primary hemostasis are usually due to?

A

Platelet Abnormalities

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13
Q

Define: Purpura

A

~3mm size of skin bleeding

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14
Q

Define: Ecchymoses

A

> 1cm size of skin bleeding

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15
Q

Having a platelet count less than this amount is associated with appearance of symptoms

A

<50,000/uL

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16
Q

This disease is an autoimmune production of IgG against platelet antigens

A

Idiopathic Thrombocytic Purpura

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17
Q

This disease is the most common cause of Thrombocytopenia in Children and Adults

A

Idiopathic Thrombocytic Purpura

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18
Q

[Pathophysiology]

Acute Idiopathic Thrombocytic Purpura

A

Autoantibodies (IgG) made in the spleen bind platelets, which are then consumed by macrophages in the spleen

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19
Q

[Treatment]

Acute Idiopathic Thrombocytic Purpura

A

Supportive

The disease is self-limiting and should resolve within weeks

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20
Q

What could you give a patient to raise platelet count with symptomatic bleeding in ITP?

A

IVIG

Short-lived effect as the spleen targets the exogenous Igs, leaving the platelet bound Igs free

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21
Q

What is the final treatment resort in cases of refractory ITP?

A

Splenectomy

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22
Q

[Pathophysiology]

Microangiopathic Hemolytic Anemia

A

Pathologic formation of platelet microthrombi in small vessels

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23
Q

Under microscopy, what is the characteristic RBC morphology when a patient has microangiopathic hemolytic anemia?

A

Schistocytes

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24
Q

Thrombotic Thrombocytopenic Purpura is caused by a decrease of what enzyme?

A

ADAMTS13

Cleaves vWF multimers into smaller monomers for degradation

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25
Q

Most common cause of TTP

A

Acquired autoantibody to ADAMTS13

Classically seen in adult females

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26
Q

Cause of Hemolytic Uremic Syndrome

A

Endothelial damage from drugs or infections

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27
Q

HUS is classically seen in which patients?

A

Children with E. coli O157:H7 Dysentery

28
Q

What does E. coli release to damage endothelial cells in HUS?

A

E. coli verotoxin

29
Q

Is the PT/PTT value elevated, unchanged, or decreased in patients with TTP/HUS?

A

Normal

30
Q

These cells undergo hyperplasia in bone marrow in patients with TTP/HUS

A

Megakaryocytes

To produce more platelets

31
Q

[Treatment]

TTP/HUS

A

Plasmapheresis and Corticosteroids

32
Q

This disease is characterized by a Genetic GP1b deficiency

What is impaired?

A

Bernard-Soulier Syndrome

Platelet adhesion is impaired

33
Q

This disease is characterized by a Genetic GIIb/IIIa deficiency

What is impaired?

A

Glanzmann Thrombasthenia

Platelet aggregation is impaired

34
Q

This drug irreversibly inactivates cyclooxygenase

A

Acetylsalicylic Acid

35
Q

End product of the coagulation cascade

A

Thrombin

36
Q

Activation of Factors of the Coagulation Cascade require?

A
  1. Exposure to activation factors
  2. Phospholipid surface of proteins
  3. Calcium
37
Q

What does PT measure?

A

Extrinsic and Common Pathways

38
Q

What does PTT measure?

A

Intrinsic and Common Pathways

39
Q

What activates Factor XII?

A

Subendothelial Collagen

40
Q

What activates Factor VII?

A

Tissue Thromboplastin

41
Q

This disease is characterized by a Genetic Factor VIII deficiency

A

Hemophilia A

42
Q

This disease is characterized by a Genetic Factor IX deficiency

A

Hemophilia B

43
Q

What is the PT/PTT findings in a patient with Hemophilia A

What is the platelet count and bleeding time?

A

Elevated PTT
Normal PT
Normal Platelet
Normal Bleeding Time

44
Q

[Treatment]

Hemophilia A

A

Recombinant Factor VIII

45
Q

Hemophilia B is also called?

A

Christmas Disease

46
Q

The most common coagulation factor inhibitor

A

Anti-FVIII

47
Q

How can you differentiate an Anti-FVIII from Hemophilia A?

A

Perform a mixing study

PTT does not correct with patient’s with Anti-FVIII

Patient’s with Hemophilia A will have PTT Correction

48
Q

This disease is the most common inherited coagulation disorder

A

Von Willebrand’s Disease

49
Q

Why do patients with Von Willebrands Disease present with increased PTT?

A

vWF stabilizes Factor VIII

50
Q

[Treatment]

Von Willebrands Disease

A

Desmopressin

Increases vWF release from Weibel-Palade bodies of endothelial cells

51
Q

Vitamin K is activated by?

A

Epoxide Reductase

52
Q

How do you test the effect of liver failure on coagulation?

A

PT

53
Q

[Mechanism of Action]

Heparin

A

Forms a Heparin-PF4 complex with platelets, allowing antibodies to be made against it leading to subsequent destruction

54
Q

What is a feared complication of Heparin-induced Thrombocytopenia?

A

Thrombosis

Results from activation of fragments of destroyed platelets

55
Q

[Pathophysiology]

Disseminated Intravascular Coagulopathy

A

Pathologic activation of the coagulation cascade causing consumption of platelets and factors as well as widespread microthrombi formation resulting in ischemia and infarction

56
Q

Common Causes of DIC

A
  1. Obstetric Complications
  2. Sepsis
  3. Adenocarcinoma
  4. Acute Promyelocytic Leukemia
  5. Rattlesnake Bite
57
Q

[Laboratory Findings]

Effect of DIC on:

  1. Platelet Count
  2. PT
  3. PTT
  4. Fibrinogen
  5. Name the elevated fibrin split product
A
  1. Decreased Platelet
  2. Elevated PT
  3. Elevated PTT
  4. Decreased Fibrinogen
  5. D-Dimer
58
Q

Best screening test for DIC

A

D-Dimer Test

59
Q

[Treatment]

Disseminated Intravascular Coagulopathy

A

Transfuse blood products and cryoprecipitate as needed

60
Q

What is responsible for cleavage of Fibrin

A

Plasmin

61
Q

What enzyme is responsible for conversion of plasminogen to plasmin

A

tPA

62
Q

[Function]

Plasmin

A
  1. Cleaves serum fibrinogen
  2. Inhibits coagulation factors
  3. Inhibits platelet aggregation
63
Q

Define: Thrombosis

A

Pathologic formation of an intravascular blood clot

64
Q

Most common location of thrombosis

A

Deep Vein of the leg below the knee

65
Q

Histologic presentation that can differentiate post from pre-mortem clots

A

Lines of Zahn

66
Q

Three Major Risk Factors for Thrombosis

A
  1. Disruption in blood flow
  2. Endothelial cell damage
  3. Hypercoagulable state

[Board Exam] Pathology (5 decks)

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