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Hematology > Hematology Week 1: Primary Hemostasis > Flashcards

Hematology Week 1: Primary Hemostasis Flashcards

1
Q

Categories of Primary hemostatic Disorders

3 listed

A
  • Disorder of platelets
  • Disorders of von Willebrand Factor
  • Disorders of connective tissue
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2
Q

Primary hemostasis Clinical Presentations

4 listed

A

Mucocutaneous hemorrhage

  • Epistaxis (nosebleeds)
  • Menorrhagia or obstetric hemorrhage
  • easy bruising, petechiae, purpura
  • prolonged bleeding after shaving or body art
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3
Q

Signs and symptoms of both primary and secondary hemostatic disorders

5 listed

A
  • Iron Deficiency Anemia (occult blood loss)
  • Bleeding after circumcision or umbilical stump bleeding
  • Dental procedure bleeding
  • History of transfusion
  • Prolonged bleeding after injuries or surgery
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4
Q

Platelet adhesion and shape change

A

platelets have folds but they bind vWF GPVI activation makes it spread out and cause platelet phospholipid spreading

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5
Q

Alpha granules # per platelet

A

40-80 per platelet

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6
Q

Alpha granules contain

4 listed

A
  • vWf
  • Factors V, VIII, and XIII
  • Fibrinogen
  • Platelet factor 4 (PF4)
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7
Q
A
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8
Q

Dense Granules # per platelet

A

4-8 per platelet

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9
Q

Dense granules contents

3 listed

A
  • stores 60-70% of platelet calcium
  • Contains ADP
  • Serotonin
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10
Q

Platelet Aggregation Process

3 listed

A
  • Glycoprotein 2b-3a primarily binds fibrinogen
  • early platelet activation opens GP 2b/3a to bind fibrinogen
  • GP 2b/3a fibrinogen further activates platelets
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11
Q

Platelet Activation: ADP Release and receptors

A

Dense granules contain ADP and platelets have ADP receptors so it self-stimulates and further activates receptors

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12
Q

Platelet ADP receptor

A

P2Y Receptor

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13
Q

Platelet Activation: Arachidonic Acid and Thromboxane Signaling

A

Arachidonic acid is converted into thromboxane A2 (TXA2) by cyclooxygenase 1 (COX-1)

TXA2 can self-stimulate and further activate other platelets

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14
Q

Laboratory tests of platelet function

4 listed

A
  • Bleeding time - time for cessation of bleeding from a standardized wound, not predictive of bleeding in non-symptomatic patients (not really used these days)
  • PFA-100 - takes patients whole blood flowing under high shear stress with agonist lined cartridges (collagen-epinephrine or collagen-ADP), platelets aggregate and occlude the aperture (closure time)
  • Platelet Aggregation - agonist addition to platelet-rich plasma to trigger platelet aggregation, light transmission increases as platelet aggregation increases
  • CBC - for platelet count
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15
Q

Bleeding Time Test

A

cut patient and time how long it bleeds

time for cessation of bleeding from a standardized wound, not predictive of bleeding in non-symptomatic patients (not really used these days)

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16
Q

PFA-100 Test

A

takes patients whole blood flowing under high shear stress with agonist lined cartridges (collagen-epinephrine or collagen-ADP), platelets aggregate and occlude the aperture (closure time)

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17
Q

Platelet Aggregation test

A

agonist addition to platelet-rich plasma to trigger platelet aggregation, light transmission increases as platelet aggregation increases

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18
Q

Glanzmann thrombasthenia is what kind of disorder?

A

Platelet aggregation disorder

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19
Q

Glanzmann thrombasthenia Clinical Features

A

Mucocutaneous Hemorrhage

Severe bleeding with trauma/surgery

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20
Q

Glanzmann thrombasthenia Genetics

A

Congenital

Autosomal Recessive

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21
Q

Glanzmann thrombasthenia Pathophysiology

A

Loss of GP2b/3a receptor = loss of platelet ability to aggregate to fibrinogen

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22
Q

Glanzmann thrombasthenia Lab Testing

A

Loss of platelet aggregation to almost all agonists

platelet counts are normal

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23
Q

Glanzmann thrombasthenia Overview

A
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24
Q

What receptor is missing in Glanzmann Thrombasthenia

A

GP 2b/3a

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25
Q

Most common Storage Pool Disorders with platelets

A

Most common is dense granule

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26
Q
A
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27
Q

Storage pool disorders Etiology

A

Often autosomal dominant

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28
Q

Clinical Presentations of Storage Pool Disorders

A

Variable degree of bleeding symptoms

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29
Q

Storage Pool Disorders pathophysiology

A

Deficiency in dense granules or granule content

30
Q

Lab testing of storage pool disorders

A
  • Decreased platelet aggregation to ADP agonist
  • Normal platelet counts
31
Q

EM of Platelet Dense Granules

A

chocolate chip vs sugar cookie

32
Q

Bernard-Soulier Syndrome Clinical Features

3 listed

A
  • Mucocutaneous Hemorrhage
  • Severe bleeding with trauma/surgery
  • Thrombocytopenia
33
Q

Bernard-Soulier Syndrome Etiology

A

Congenital

Autosomal Recessive

34
Q

Bernard-Soulier Syndrome Pathophysiology

A

Defective or absent GP1b Complex

35
Q

Bernard-Soulier Syndrome Lab Testing

A
  • Low platelet count (<150,000/mm3)
  • Normal platelet aggregation to almost all agonists
36
Q

Bernard-Soulier Syndrome Overview

A
37
Q

Bernard-Soulier Syndrome is a disorder of?

A

Platelet adhesion and thrombocytopenia

38
Q

What is missing in Bernard-Soulier Syndrome

A

GP1b complex

39
Q

thrombocytopenia definition

A

platelet count of 150,000/mm3

40
Q

Thrombocytopenia Mechanisms

3 listed

A
  • production failure
  • destruction
  • sequestration
41
Q

Acquired Production failure mechanism of thrombocytes

4 listed

A
  • Aplastic anemia
  • myelosuppressive therapy (i.e. chemotherapy for leukemia)
  • Viral infection (HIV, EBV, CMV, Rubella, and more)
  • ethanol abuse
42
Q

Congenital Production failure mechanism of thrombocytes

A

Bernard-Soulier Syndrome (congenital thrombocytopenia)

43
Q

Categories of Platelet destruction mechanisms of thrombocytopenia

2 listed

A

Immune Mediated

Non-immune mediated

44
Q

Immune Mediated Platelet destruction mechanisms of thrombocytopenia

3 listed

A
  • Immune thrombocytopenic purpura (ITP)
  • Infection (HIV, HepC, Helicobacter pylori)
  • Autoimmune disorders (SLE)
45
Q

Non-Immune Mediated Platelet destruction mechanisms of thrombocytopenia

4 listed

A
  • Disseminated intravascular coagulation (DIC)
  • Drug-induced thrombocytopenia (i.e. quinine)
  • Sepsis
  • Thrombotic microangiopathies (i.e. TTP, Hemolytic uremic syndrome)
46
Q

Quinine was used for?

A

To prophylactically treat malaria

47
Q

ITP AKA

A

Immune Thrombocytopenic Purpura

48
Q

ITP Clinical presentations

A
  • isolated thrombocytopenia with no apparent acquired or congenital cause for thrombocytopenia
  • Diagnosis of exclusion
49
Q

ITP Platelet Counts

A

range from mild to severe thrombocytopenia

50
Q

ITP Pathophysiology

A
  • Increased platelet destruction by antiplatelet antibodies
  • suppressed platelet production
  • can also target megakaryocytes preventing platelet production
51
Q

Acute ITP

A
  • Rapid onset of bleeding symptoms
  • Most common form of ITP; typically seen in children < 10 years old
  • Spontaneous resolution in <3 months is typical
  • Conservative treatment: watchful waiting vs pharmacologic treatment
52
Q

Chronic ITP

A
  • Typically seen in adults
  • can evolve from acute ITP in children
  • insidious onset with persistent thrombocytopenia
53
Q

Acute vs Chronic ITP

A
54
Q

ITP Therapies

A
  • Intravenous Immune Globulin (IVIg)
  • Glucocorticoids
  • Splenectomy
55
Q

Platelet Sequestration due to splenomegaly mechanism of thrombocytopenia

A

seen in 2 conditions

  • lymphomas
  • Cirrhosis (portal hypertension leading to splenomegaly)

Normal sized spleen holds 1/3 circulating platelets

Severe splenomegaly can result in platelet counts < 50,000/mcl

56
Q

vWF AKA

A

von Willebrand Factor

57
Q

vWF binds?

A
58
Q

vWF is synthesized by?

A

Endothelial cells and megakaryocytes

59
Q

What is vWF?

A

forms long multimers which binds platelet GP1b

is a carrier protein for factor 8

60
Q

vWF functions

A

platelet adhesion to vessel wall

Shear-induced platelet aggregation

Carrier for factor 8

61
Q

Which vWF multimers have the greatest affinity for GP1b

A

longer vWF multimers have greater GP1b binding affinity

62
Q

VWD AKA

A

Von Willebrand Disease

63
Q

VWD types

3 listed

A

type 1

Type 2

Type 3

64
Q

Type 1 VWD

A

lower vWF levels

65
Q

Type 2 VWD

A

functional vWF abnormality

66
Q

Type 3 VWD

A

vWF is virtually absent

67
Q

What is the most common congenital bleeding disorder?

A

VWD

1% of the population has VWD

68
Q

VWD principles of therapy

A

Dual-defect of hemostasis

Lab test of vWF activity do not always predict the bleeding

69
Q

VWD Therapy

A

Desmopressin (DDAVP)

70
Q

DDAVP AKA

A

Desmopressin

71
Q

DDVAVP used to treat?

A

VWD

72
Q

DDAVP is?

A
  • a synthetic analog of vasopressin without the vasopressor activity
  • most useful type 1 vWD
  • I.V. and nasal spray
  • Mechanism: triggers release of vWF from Weibel-Palade bodies in endothelial cells

Hematology (26 decks)

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