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Hematology 2 > Lecture - Mixed > Flashcards

Lecture - Mixed Flashcards

1
Q

The maintenance of circulatory hemostasis is achieved through the process of balancing

A

bleeding (hemorrhage)

clotting (thrombosis)

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2
Q

4 Major Components of Hemostasis

A

Vascular system
Thrombocytes (platelets)
Blood Coagulation Factors
Ultimate tissue repair

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3
Q

Processes involved in hemostasis following injury to a small blood vessel:

A
  1. Blood vessel spasm
  2. Formation of a platelet plug
  3. Contact among damaged blood vessel, blood platelet, and
    coagulation proteins
  4. Development of a blood clot around the injury
  5. Fibrinolytic removal of excess hemostatic material to
    reestablish vascular integrity
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4
Q

Types of Blood Vessels

A

Arteries - arterioles
Veins - venules
Capillaries

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5
Q

Tissue Zones

A
  1. Tunica adventitia / Tunica
    Externa
  2. Tunica media
  3. Tunica intima / Tunica interna
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6
Q

a reflex in which blood vessels narrow to increase blood pressure.

A

Vasoconstriction

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7
Q

Vasoconstriction caused by

A

thromboxane A2

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8
Q

promotes vasoconstriction

A

Epinephrine

serotonin

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9
Q

ensures rapid lysis of fibrin clots

A

plasminogen

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10
Q

Regulates the permeability of the inner vessel wall and provides the principal stimulus to thrombosis following injury to a blood vessel.

A

Endothelium

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11
Q

Involved in the clotting process by producing or storing clotting components

A

Endothelium

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12
Q

Rich with plasminogen activator, which, if appropriately stimulated, is released and activates plasminogen

A

Endothelium

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13
Q

synthesized by the endothelium from prostaglandin precursors and strongly inhibits platelet aggregation and adhesion

A

prostacyclin

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14
Q

where they act as modulators of vasomotor tone, cell proliferation, and hormone production

A

Endothelins

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15
Q

Endothelin-1 is produced in

A

endothelial cells

vascular smooth muscle cells

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16
Q

endothelin acts as modulator in

A

vasomotor tone
cell proliferation
hormone production

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17
Q

Endothelin-2 produced in

A

predominantly within the kidney and intestine

smaller amounts produced in the myocardium, placenta, and uterus

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18
Q

Endothelin-3 found in

A

high concentrations in the brain - proliferation and development in neurons and astrocytes

found throughout the gastrointestinal tract and in the lung and kidney.

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19
Q

play an important role in the initiation, progression, and clinical complications of various forms of inflammatory and degenerative vascular diseases.

A

Endothelial Dysfunction

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20
Q

Stimuli of Endothelial Dysfunction

A

Immunoregulatory Substances (TNF & IL-1)
Viral Infection and Transformation
Bacterial Toxins
Cholesterol / Oxidatively modified lipoproteins

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21
Q

Disruption of the endothelium directly activates all four components of hemostasis.
• After this event, the following events take place:

A
  1. Initially, rapid vasoconstriction for up to 30 minutes reduces blood flow and promotes contact activation of platelets and coagulation factors.
  2. In the second phase, platelets adhere immediately to the exposed subendothelial connective tissue, particularly collagen. The aggregated platelets enhance sustained vasoconstriction by releasing thromboxane A2 and vasoactive amines, including serotonin and epinephrine.
  3. In the third phase, coagulation is initiated through both the intrinsic and extrinsic systems.
  4. Finally, fibrinolysis occurs following the release of tissue
    plasminogen activators (t-PAs) from the vascular wall. Fibrinolytic removal of excess hemostatic material is necessary to reestablish vascular integrity.
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22
Q

Essential Factors for Vascular Integrity

A
  1. Circulating functional platelets
  2. Adrenocorticosteroids
  3. Ascorbic Acid
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23
Q

The integrity of arterioles and venules depends on

A

vasoconstriction
platelet plug
fibrin clot

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24
Q

ultimate importance in damaged arteries

A

Vasoconstriction

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25
Q

Veins contain what percent of blood volume

A

70%

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26
Q

Veins, which contain 70% of the blood volume, may rupture with

A

slight increase in hydrostatic pressure

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27
Q

proceeds initially through a phase characterized by mitotic division of a progenitor cell, followed by a wave of nuclear endoreduplication.

A

Megakaryocytopoiesis

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28
Q

proceeds initially through a phase
characterized by mitotic division of a progenitor cell,
followed by a wave of nuclear endoreduplication

A

Megakaryocytopoiesis

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29
Q

the process in which chromosomal material (DNA) and the other events of mitosis occur

A

Endoreduplication

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30
Q

stimulate the production and maturation of megakaryocytes

A

Thrombopoietin

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31
Q

Thrombopoietin activity results from several different cytokines

A

erythropoietin
IL-3
granulocyte-macrophage colony-stimulating factor (GM-CSF)

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32
Q

erythropoietin, IL-3, and granulocyte-macrophage colony-stimulating factor (GM-CSF) have been shown to be able to increase

A

megakaryocyte size
maturational stage
ploidy

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33
Q

10-24 mm, round nucleus, N/C ratio 3:1

A

MEGAKARYOBLAST

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34
Q

chromatin of megakaryoblast

A

homogenous

loosely organized

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35
Q

granules are absent by wright’s stain

A

megakaryoblast

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36
Q

Bone Marrow: 20% of megakaryocyte precursors in bone marrow

Peripheral Blood: 0%

A

megakaryoblast

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37
Q

15-40 mm, indented nucleus, condensed chromatin

A

promegakaryocyte

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38
Q

granules are present, n/c ration 1:2

A

PROMEGAKARYOCYTE

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39
Q

REFERENCE INTERVAL:
Bone Marrow: 25% of megakaryocyte precursors in bone marrow
Peripheral Blood: 0%

A

PROMEGAKARYOCYTE

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40
Q

largest bone marrow cells, ranging up to 160 mm in size

A

MEGAKARYOCYTE

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41
Q

MEGAKARYOCYTE n/c ratio

A

1:12

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42
Q

multilobular, not multinucleated

A

megakaryocyte

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43
Q

2 to 4 mm

no nucleus

A

platelets

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44
Q

Platelet’s color of cytoplasm and granules

A

cytoplasm - light blue

granules - fine red-purple

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45
Q

appearance of inactive or unstimulated platelet

A

thin

smooth surfaced disc

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46
Q

alteration in cellular shape of platelet is triggered by

A

cytoplasmic calcium

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47
Q

Stronger stimulation causes platelets to

A

become sticky without losing their discoid shape

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48
Q

GLYCOCALYX also known as

A

fluffy coat

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49
Q

these surrounds the cellular membrane of the platelet externally.

A

glycocalyx or fluffy coat

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50
Q

unique among the cellular components of the blood

A

glycocalyx or fluffy coat

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51
Q

glycocalyx or fluffy coat is composed of

A

plasma proteins and carbohydrate molecules

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52
Q
  • located directly beneath the cell membrane of platelet
  • provide structure of the platelet to maintain its discoid shape
  • maintains the position of the organelles
A

MICROFILAMENTS & MICROTUBULES

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53
Q

• secondary system of microfilaments is functional in

A

internal organization

secretion of blood coagulation products, such as fibrinogen

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54
Q

most abundant granules

A

alpha

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55
Q

enumerate alpha granules

A 
heparin-neutralizing platelet factor 4
beta-thromboglobulin
platelet-derived growth factor
platelet fibrinogen
fibronectin
vwf
thrombospondin
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56
Q

dense granules also known as

A

delta granules

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57
Q
  • store hydrolase enzymes
A

lysosomes

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58
Q

Dense or Delta Granules contains

A

Serotonin
ADP
ATP
Calcium

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59
Q

Other Cytoplasmic Constituents

A
  • Contractile proteins, including actomyosin (thrombosthenin), myosin, and filamin
  • Glycogen
  • Enzymes of the glycolytic and hexose pathways
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60
Q

megakaryocyte produces

A

1,000-2,000 platelets

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61
Q

Marrow transit time / maturation period of megakaryocyte

A

5 days

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62
Q

Platelets initially enter the spleen & remains for

A

2 days

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63
Q

_____ of the total number of platelets are in systemic circulation

A

2/3

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64
Q

____ exists as pool of platelets in the spleen that free exchange with the general circulation

A

1/3

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65
Q

A normal person has an average of ____ platelets in the systemic
circulation.
Range:

A

250 × 109/L

150 ×109/L to 450 × 109/L

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66
Q

Platelet turnover or effective thrombopoiesis averages

A

35 ×109/L ± 4.3 × 109/L/day

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67
Q

The life span of a mature platelet is

A

9.0 days ± 1 day

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68
Q

At the end of their life span, platelets are phagocytized by the

A

liver and spleen

mononuclear phagocytic system

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69
Q

2 important players in primary hemostasis

A

blood vessels

platelets

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70
Q

Largest artery

A

aorta

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71
Q
  • if blood comes out from vessel, instead of closed system what will happen
A

formation of petechiae
purpura
ecchymoses

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72
Q
  • noncontact surface in vivo
A

Endothelium

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73
Q

contact surface, when exposed hemostatic mechanism will start

A

Sub endothelium

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74
Q

involved in secondary hemostasis

A

Blood coagulation factors

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75
Q

Mechanism of hemostasis only works on

A

medium sized vessels

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76
Q
  • end point of primary hemostasis
A

Formation of platelet plug

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77
Q

doesn’t have tunic

A

Capillaries

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78
Q

epithelium of Surface endothelium

A

simple squamous

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79
Q

found in tunica media

A

collagen/smooth muscles

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80
Q

vasobasorum

A

blood vessels that gives nourishment and blood to blood vessels

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81
Q

vasobasorum found in

A

tunica externa

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82
Q
  • product of activated platelet that will prolong platelet adhesion and vasoconstriction, byproduct of eicosanoids or the thromboxane pathway of cyclooxygenase pathway
A

Thromboxane A2

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83
Q
  • only interleukins secreted by monocyte and macrophage
A

Tumor necrosis factor/IL1

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84
Q

these makes collagen

A

fibroblasts

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85
Q

endo multiplication of DNA, only lobes increase called polyploid cells

A

Endomitosis

86
Q

the normal percentage of megakaryocytic cells are found in the bone marrow

87
Q

The earliest recognizable by microscopy

A

megakaryoblast

88
Q

biggest in bone barrow or mother cell of platelets

A

Meta mega/mature karyocyte

89
Q

no cytokinesis or late telophase, formation of?

A

Polyploids

90
Q

simple endothelial cells that lines delicate organs like liver, spleen, BM

91
Q

Sinusoids lines delicate organs like

A

BM
liver
spleen

92
Q

Platelet will stay at sinusoids for

A

9-11 days or 8-10 days

93
Q

has big role on erythropoiesis and has a big role on thrombopoiesis

94
Q

pan myeloid factor, affects all cell line

A

• GM-CSF

95
Q

more ploidy

A

greater number of lobes

96
Q

how to get chromosome numbers

97
Q

Megakaryocytes are not usually found in blood, what happens if there are some?

A

megakaryocytic leukemia

98
Q

Do we find platelets in bone marrow?

99
Q

Most important criterion in determining the age of the cell

A

chromosome- fine and linear

100
Q

Platelets take what dyes

A

azure dyes

101
Q

zone for adhesion of platelets

A

peripheral zone

102
Q

zone where actomyosin or thrombosthenin, microfilaments, microtubules are found

A

Sol-gel zone

103
Q

responsible for disk shape

A

microtubules

104
Q

Not greatly stainable under electron micrographs they are gray colored

A

Alpha granules

105
Q

Platelets can do aerobic glycolysis due to presence of

A

mitochondria

106
Q
  • an internal membrane structure found in platelets
A

Canalicular system

107
Q

Adhesion is direct or indirect

108
Q

vWF produced in

A

Weibel-palate bodies

109
Q

Weibel-palate bodies found in

110
Q

these also produce vWF aside from Weibel-palate

A

Megakaryocytes

111
Q

3 primary steps in hemostasis

A

Adhesion
secretion (release mechanism)
aggregation

112
Q

Upon exposure of sub endothelium, this will happen

113
Q

a loose mass (mesh) that will be strengthened by secondary hemostasis

A

Platelet plug formation

114
Q

glycoproteins attached on surface of platelets

A

GP1B
GP5
GP9

115
Q

this will engender (cause) platelet aggregation

116
Q

receptors for fibrin

117
Q

Functional platelets adhere to

A

subendothelial collagen

118
Q

these are assessed using platelet aggregometry

A

Platelet adhesion, aggregation, and secretion

119
Q

PRP aggregometry is performed using

A

a light-transmittance aggregometer

120
Q

whole-blood platelet aggregometry, platelet aggregation is measured by

A

electrical impedance.

121
Q

used for the simultaneous measurement of platelet aggregation and the secretion of adenosine triphosphate (ATP) from activated platelet dense granules

A

Whole Blood/Optical LumiAggregometer

122
Q

these are used to test for abnormalities of specific membrane binding sites.

A

platelet agonists

123
Q

Aggregation is dependent in

A

calcium and energy (ATP)

124
Q

Aspirin (blood thinner) will block

A

cyclooxygenase
thromboxane
eicosanoid
prostaglandin pathways

to stop production of thromboxane A2

125
Q

potent platelet aggregator and stimulator

A

Thromboxane A2

126
Q

blood clot, aggregates of platelets superimposed by fibrin

127
Q

Secondary hemostasis endpoint

A
  • stable fibrin clot in vivo (fibrin polymer)
128
Q

Electronic particle counter principle

A

coulter principle

works in electrical impedance

129
Q

Template bleeding time

A

obsolete, used to be the gold standard

130
Q

When antibody coats a particle, it will activate

A

classical pathway of complements that will result in lysis of platelets

131
Q

Not enough glycoproteins 1B, 5 and 9

A

Bernard-Soulier syndrome

132
Q

Not enough glycoproteins 2b, 3a

A

Glanzmann diseases

133
Q

Defect usually is in

A

release mechanism

134
Q
  • reactive condition
A

thrombocytosis

135
Q

release of Pf3 or PPL(phospholipid)- which plays a big role in forming stable fibrin clot.

A

secondary hemostasis

136
Q

substance responsible for extrusion of serum from the clot - clot retraction

A

platelet contractile protein, THROMBOSTHENIN/ACTOMYOSIN

137
Q

plays a big role in forming stable fibrin clot in secondary hemostasis

A

Pf3 and phospholipid

138
Q

Functions of platelets

A

Primary hemostasis
Secondary hemostasis
Clot retraction
Localization of bacteria and other small objects

139
Q

Platelets are composed of

A

60% proteins
30% lipids
8% carbohydrate
Mainly glycogen, sulfate mucopolysaccharides, various minerals, water and nucleotides
Lipids- phosphatidylethanolamine and phosphatidylserine

140
Q

4 parts of platelet

A

Peripheral zone
Sol-gel zone
Organelle zone
Membranous system

141
Q

the fuzzy coating, primarily composed of glycoproteins (GP)

A

Glycocalyx

142
Q

receptors for vWF

143
Q

major component of plasma membrane

A

arachidonic acid

144
Q

lies directly beneath the glycocalyx and is composed of asymmetrically distributed phospholipids embedded with integral proteins

A

plasma membrane

145
Q

site where messages from the external membrane are translated into chemical signals causing activation and a physical change in the platelets.

A

sub membranous area

146
Q

receptors identified in sub membranous area

A 
ADP
collagen
serotonin
epinephrine
thrombin
vWF
Factor V
Factor Xa
147
Q

zone that lies beneath the plasma membrane.

A

Sol-gel zone

148
Q

Sol-gel zone is composed of

A

Microfilaments and microtubules

149
Q

responsible for clot retraction

A

actomyosin (thrombosthenin)

150
Q

maintains the platelet’s disc shape

A

microtubules - tubulin

151
Q

called the centromere

A

Organelle zone

152
Q

Part where the secretory products come from.

A

Organelle zone

153
Q

play an important role in platelet aggregation especially its product, ADP

A

Dense/delta granules

154
Q

The platelets can also do anaerobic glycolysis due to

A

glycogen granules

155
Q

2 types of membranous system

A

dense tubular system

surface connected system

156
Q

derived from smooth endoplasmic reticulum and sequesters calcium for platelet activation processes.

A

dense tubular system-

157
Q

an invagination of the plasma membrane, acts as a canal for the release of the granule contents and cytoplasm to the exterior of the platelet

A

surface connected system

158
Q

This system is also involved in platelet phagocytosis.

A

surface connected system

159
Q

This is found in hemorrhagic diathesis(( bleeding tendency)

A

Hemmeler’s anomaly

Hermansky-pudlak syndrome

160
Q

defect when no azurophilic granules is found in cytoplasm of

platelets. The absolute platelet count is within reference range.

A

Hemmeler’s anomaly

161
Q

Platelets have storage pool deficiency.

A

Hermansky-Pudlak syndrome

162
Q

found in albinos , there is hemorrhagic diathesis, dense bodies storage pool disease

A

Hermansky-Pudlak syndrome-

163
Q

appearance of atypical platelet

A

giant 4-7 um
increase size of hyalomere
granule is decreased or absent
bizarre or irregular shapes

164
Q
  • defects where there is decreased number of alpha granules
A

Gray platelet syndrome

165
Q

when platelets are found encircling the borders of neutrophils, seen in patients whose blood was anticoagulated with EDTA

A

Platelet satellitosis/ platelet satellitism

166
Q

This phenomenon is thought to be due to a serum factor that reacts in the presence of EDTA.

A

Platelet satellitosis/ platelet satellitism

167
Q

occurs within the intravascular

compartment lined with endothelium

A

HEMOSTASIS

168
Q

A tightly regulated balance between formation and dissolution of hemostatic plugs modulated by a series of enzymes and scaffolding proteins.

A

PHYSIOLOGIC HEMOSTATIC SYSTEM

169
Q

Factors involved in Normal

Hemostasis and Thrombosis

A

Plateletes, Granulocytes & Monocytes

Coagulation Protein Sytem (Clot Forming)

Fibrinolysis Protein System (Clot Lysing)

Anticoagulation Protein System ( Regulating)

170
Q

serves to form thrombin that initiates the proteolysis of fibrinogen, leading to fibrin clot formation

A

Coagulation System (Clot Forming)

171
Q

→ functions to lyse the clot formed by thrombin

A

Fibrinolytic Sytem (Clot Lysing)

172
Q

regulates all enzymes of the coagulation and fibrinolytic systems, so that no inappropriate excess of clotting or bleeding occurs.

A

Anticoagulation System (Regulating)

173
Q

Vasoconstriction is a short-lived reflex reaction of the smooth muscle in the vessel wall produced by the

A

sympathetic branches of the autonomic nervous system

174
Q

increases the adhesiveness of platelets

175
Q

Platelet during adhesion will transform from ________ to ______ which produces surface membrane organization

A

disc shape to a sphere with pseudopods

176
Q

Internal contraction of the platelet results in release of granular contents of

A

alpha granules
dense granules
lysosomal granules

177
Q

Platelets adhere at sites of mechanical vascular injury and then undergo activation and express what functional glycoprotein —- also referred to as?

A

IIb/IIIa

Alpha 2b Beta 3

178
Q

Which receptors will mediate the recruitment of local platelets by forming fibrinogen bridges between platelets—a process called platelet cohesion.

A

glycoprotein IIb/IIIa

179
Q

A process forming fibrinogen bridges between platelets

A

platelet cohesion

180
Q

Glycoprotein ____ is specific for

platelets.

181
Q

Glycoprotein ______ is the most abundant platelet membrane protein (with approximately _______ receptors per platelet).

A

IIb/IIIa

50,000

182
Q

gold standard test to determine platelet function.

A

Platelet aggregation

183
Q

Agents capable of producing platelet aggregation in vitro (an energy dependent process):

A

Collagen

Proteolytic enzymes (thrombin)

Biological amines (epinephrine and serotonin)

184
Q

results from bridges formed by fibrinogen in the presence of calcium which produces sticky surface on platelets

A

Aggregation

185
Q

If these aggregates are reinforced by fibrin, they are referred to as a

186
Q

Aggregation of platelets by at least one pathway can be blocked by substances such as:

A

PGE (prostaglandin E)

adenosine

187
Q
  • provides basis for platelet consolidation & stabilization.
A

Fibrinogen

188
Q

This process involves the precipitation of polymerized fibrin around each platelet

A

PLATELET PLUG CONSOLIDATION & STABILIZATION

189
Q

Result of platelet plug consolidation and stabilization

A

fibrin clot

190
Q

Fibrin clot will produce

A

irreversible platelet plug

191
Q

Quantitative Determination of Platelet

A

Electronic particle counter

examination of stained blood film

192
Q

done if the platelet count is normal → assessment of platelet function

A

Qualitative Assessment of Platelet

193
Q

Qualitative Assessment of Platelet

A

a. PBS
b. Platelet Count
c. Template Bleeding Time
d. Petechiometer
e. Platelet Aggregation
f. Platelet lumiaggregation (release)
g. Platelet Antibodies (IgM and IgG)
h. Platelet Membrane Glycoproteins
i. Platelet factor IV
k. Thromboxanes

194
Q

In vivo measurement of platelet adhesion and aggregation on locally injured vascular subendothelium.

A

Bleeding Time with and without Aspirin

195
Q

Provides an estimate of the integrity of the platelet plug and thereby measures the interaction between the capillaries and platelets

A

Bleeding Time with and without Aspirin

196
Q

Reflects the number and quality of platelets, fibrinogen concentration, fibrinolytic activity, and packed red cell volume.

A

Clot Retraction

197
Q

The degree of clot retraction is __________ to the number of

platelets & __________ to the hematocrit and the level of the blood coagulation factor fibrinogen.

A

directly proportional

inversely proportional

198
Q

The principle of the test is that platelet-rich plasma is treated with a known aggregating agent. If aggregated, cloudiness or turbidity can be measured using a spectrophotometer.

A

Platelet Aggregation

199
Q

Aggregating agents

A 
ADP
Collagen
Epinephrine
Ristocetin
Snake venom
Thrombin
200
Q

Available techniques can include complement fixation methods, lysis of chromium 51 labeled platelets, assays of platelet-bound immunoglobulins, and competitive inhibition assays.

A

Antiplatelet Antibody Assays

201
Q

VASCULAR DISORDERS

A
  1. Purpura associated with direct endothelial cell damage.
  2. Purpura associated with an inherited disease of the connective tissue.
  3. Purpura associated with decreased mechanical strength of the microcirculation.
  4. Purpura associated with mechanical disruption of small venules.
  5. Purpura associated with microthrombi (small clots).
  6. Purpura associated with vascular malignancy.
202
Q

Demonstrates the smallest platelets seen

A

Wiskott-Aldrich

syndrome

203
Q

Characterized by the presence of large platelets and the presence of Döhle-like bodies in the granulocytic leukocytes

A

May-Hegglin

anomaly

204
Q

Disorder that exhibits giant platelets and thrombocytopenia

A

Alport syndrome

205
Q

Demonstrates the largest platelets. In this disorder, it has been demonstrated that the giant platelets are probably an artifact of the slide

A

Bernard-Soulier syndrome (giant platelet syndrome)

206
Q

3 types of thrombocytopenia

A

Disorder of production
disorder of destruction or utilization
disorder of platelet distribution

207
Q

Hereditary or familial thrombocytosis associated with ger_x0002_mline mutations of the thrombopoietin (THPO) gene in the THPO receptor (MPL) gene

A

THROMBOCYTOSIS

208
Q

associated with myeloproliferative neoplasms and/or myelodysplastic disorders (clonal thrombocytosis associated with somatic mutations of JAK2[V617F], MPL, and additional currently unknown genes)

A

THROMBOCYTOSIS

209
Q

reactive disease

A

secondary thrombocytosis

210
Q

TYPES OF PLATELET DYSFUNCTION

A

a) Acquired
b) Drug-Induced
c) Platelet Membrane Receptors
d) Hereditary

211
Q

Types of acquired platelet dysfunction

A 
Myeloproliferative
Uremia
Miscellaneous 
Paraprotein
Cardiopulmonary bypass and platelet function
212
Q

Types of hereditary platelet dysfunction

A

i. Bernard-Soulier Syndrome
ii. Glanzmann Thrombasthenia and Essential Athrombia
iii. Hereditary Storage Pool Defect

Hematology 2 (8 decks)

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