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Hematology > Hematology Week 1: Primary Hemostasis > Flashcards

Hematology Week 1: Primary Hemostasis Flashcards

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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
Most common Storage Pool Disorders with platelets
Most common is dense granule
26
27
Storage pool disorders Etiology
Often autosomal dominant
28
Clinical Presentations of Storage Pool Disorders
Variable degree of bleeding symptoms
29
Storage Pool Disorders pathophysiology
Deficiency in dense granules or granule content
30
Lab testing of storage pool disorders
* Decreased platelet aggregation to ADP agonist * Normal platelet counts
31
EM of Platelet Dense Granules
chocolate chip vs sugar cookie
32
Bernard-Soulier Syndrome Clinical Features 3 listed
* Mucocutaneous Hemorrhage * Severe bleeding with trauma/surgery * Thrombocytopenia
33
Bernard-Soulier Syndrome Etiology
Congenital Autosomal Recessive
34
Bernard-Soulier Syndrome Pathophysiology
Defective or absent GP1b Complex
35
Bernard-Soulier Syndrome Lab Testing
* Low platelet count (\<150,000/mm3) * Normal platelet aggregation to almost all agonists
36
Bernard-Soulier Syndrome Overview
37
Bernard-Soulier Syndrome is a disorder of?
Platelet adhesion and thrombocytopenia
38
What is missing in Bernard-Soulier Syndrome
GP1b complex
39
thrombocytopenia definition
platelet count of 150,000/mm3
40
Thrombocytopenia Mechanisms 3 listed
* production failure * destruction * sequestration
41
Acquired Production failure mechanism of thrombocytes 4 listed
* Aplastic anemia * myelosuppressive therapy (i.e. chemotherapy for leukemia) * Viral infection (HIV, EBV, CMV, Rubella, and more) * ethanol abuse
42
Congenital Production failure mechanism of thrombocytes
Bernard-Soulier Syndrome (congenital thrombocytopenia)
43
Categories of Platelet destruction mechanisms of thrombocytopenia 2 listed
Immune Mediated Non-immune mediated
44
Immune Mediated Platelet destruction mechanisms of thrombocytopenia 3 listed
* Immune thrombocytopenic purpura (ITP) * Infection (HIV, HepC, Helicobacter pylori) * Autoimmune disorders (SLE)
45
Non-Immune Mediated Platelet destruction mechanisms of thrombocytopenia 4 listed
* Disseminated intravascular coagulation (DIC) * Drug-induced thrombocytopenia (i.e. quinine) * Sepsis * Thrombotic microangiopathies (i.e. TTP, Hemolytic uremic syndrome)
46
Quinine was used for?
To prophylactically treat malaria
47
ITP AKA
Immune Thrombocytopenic Purpura
48
ITP Clinical presentations
* isolated thrombocytopenia with no apparent acquired or congenital cause for thrombocytopenia * Diagnosis of exclusion
49
ITP Platelet Counts
range from mild to severe thrombocytopenia
50
ITP Pathophysiology
* Increased platelet destruction by antiplatelet antibodies * suppressed platelet production * can also target megakaryocytes preventing platelet production
51
Acute ITP
* 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
Chronic ITP
* Typically seen in adults * can evolve from acute ITP in children * insidious onset with persistent thrombocytopenia
53
Acute vs Chronic ITP
54
ITP Therapies
* Intravenous Immune Globulin (IVIg) * Glucocorticoids * Splenectomy
55
Platelet Sequestration due to splenomegaly mechanism of thrombocytopenia
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
vWF AKA
von Willebrand Factor
57
vWF binds?
58
vWF is synthesized by?
Endothelial cells and megakaryocytes
59
What is vWF?
forms long multimers which binds platelet GP1b is a carrier protein for factor 8
60
vWF functions
platelet adhesion to vessel wall Shear-induced platelet aggregation Carrier for factor 8
61
Which vWF multimers have the greatest affinity for GP1b
longer vWF multimers have greater GP1b binding affinity
62
VWD AKA
Von Willebrand Disease
63
VWD types 3 listed
type 1 Type 2 Type 3
64
Type 1 VWD
lower vWF levels
65
Type 2 VWD
functional vWF abnormality
66
Type 3 VWD
vWF is virtually absent
67
What is the most common congenital bleeding disorder?
VWD 1% of the population has VWD
68
VWD principles of therapy
Dual-defect of hemostasis Lab test of vWF activity do not always predict the bleeding
69
VWD Therapy
Desmopressin (DDAVP)
70
DDAVP AKA
Desmopressin
71
DDVAVP used to treat?
VWD
72
DDAVP is?
* 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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