3RD HEMA - PART 4 (Hemostasis, Megakaryocytopoiesis) Flashcards

1
Q

Maintenance of blood flow within the vascular system.

A

HEMOSTASIS

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2
Q

Involves the following:
● Constriction of damaged blood
vessels
● Formation of platelet plugs

A

PRIMARY HEMOSTASIS

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3
Q

(2) Major participants of PRIMARY HEMOSTASIS

A

● Blood vessels
● Platelets

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4
Q

Involves the following:
● Formation of fibrin meshwork through activated coagulation
factors
● Inhibition of activated coagulation factors

A

SECONDARY HEMOSTASIS

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5
Q

Aka: thrombocytes

A

PLATELETS

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6
Q

Arise from a bone marrow cell called

A

megakaryocytes

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7
Q

● Important in both primary and
secondary hemostasis
● Described as cells with granular
cytoplasm but no clear material

A

PLATELETS

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8
Q

● __________ cluster with the RBCs near the center of the blood vessel
● _____________ move back and forth with the WBCs from the venules into the white pulp of the spleen

A

PLATELETS

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9
Q

Lifespan of the Platelets

A

9 days +/- 1 day
(8-10 days)

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10
Q

On wright-stained PBS
● Spread throughout the RBC
monolayer (7-21 cells per 100x field)
● Average diameter of 2.5um (or, 2-4 um)

A

PLATELETS

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11
Q

Aka stress platelets

A

RETICULATED PLATELETS

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12
Q

● Appear in compensation for
thrombocytopenia
● Newly released from megakaryocytes and still contain RNA

A

RETICULATED PLATELETS

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13
Q

● Potentially prothrombotic (may be associated with increased risk of cardiovascular disease)

A

RETICULATED PLATELETS

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14
Q

● Early predictor of bone marrow
recovery after chemotherapy and
transplantation

A

RETICULATED PLATELETS

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14
Q

● Markedly larger than the usual
platelets (diameter in PBS: exceeds 6um [MPV reaches 12 to 14 fL])

A

RETICULATED PLATELETS

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15
Q

● Can help differentiate bone marrow failure from peripheral destruction in thrombocytopenia

A

RETICULATED PLATELETS

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16
Q

It is believed that thrombocytes enter the ____ initially, where they remain for ____ days. After this period, thrombocytes are in either the __________ blood or in the active splenic pool

A

It is believed that thrombocytes enter the spleen initially, where they remain for two days. After this period, thrombocytes are in either the circulating blood or in the active splenic pool

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17
Q

SIZE OF THE NORMAL PLATELETS

Diameter:

A

2.5um (average)

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17
Q

Approximately __ of the total number of platelets are in the systemic circulation, while ___ of the platelets are in the spleen.

A

Approximately ⅔ of the total number of platelets are in the systemic circulation, while ⅓ of the platelets are in the spleen.

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18
Q

SIZE OF THE NORMAL PLATELETS

Mean platelet volume (MPV):

A

Reference range: 6.8 to 10.2 fL

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19
Q

_______ causes swelling of platelets (causes approximately 20% increase in MPV during the first hour)

A

EDTA

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20
Q

Should not be based on EDTA specimens that are between ___ to ___ hours old

A

1 to 4 hours old

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21
Q

If the physician is looking for the
MPV value you need to wait first for ___ hour

A

1 hour

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22
Q

According to Turgeon, if you measure the ____ right away, there might be a mistake because the platelets are still swollen.

A

MPV

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23
Q

How many platelets are to be found on a normal blood smear?

a. 7-21 platelets per 10x field
b. 7-21 platelets per 100 x field

A

7-21 platelets per 100 x field

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24
Q

What is the other name for reticulated platelet? (from rodak’s)

A

Stress platelets

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25
Q

EXAMPLES OF DISORDERS CHARACTERIZED BY SIZE

SMALL PLATELETS

(2)

A
  1. Wiskott - Aldrich Syndrome (WAS)
  2. TORCH infections
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26
Q

EXAMPLES OF DISORDERS CHARACTERIZED BY SIZE

LARGE / GIANT PLATELETS

(3)

A
  1. Bernard-Soulier Syndrome
  2. Gray Platelet Syndrome
  3. May - Hegglin Anomaly
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27
Q

Genetic inheritance of WISKOTT - ALDRICH SYNDROME (WAS)

A

X - linked recessive

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28
Q

Was also a White Blood Cell anomaly

A

WISKOTT - ALDRICH SYNDROME (WAS)

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29
Q

Triad of WAS: [TIE]

A

● Thrombocytopenia
● Immunodeficiency
● Eczema

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30
Q

Decreased platelet count

A

Thrombocytopenia

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31
Q

The patient also sufferers
from impaired function of
WBCs

A

Immunodeficiency

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32
Q

Genetic inheritance of BERNARD - SOULIER SYNDROME (BSS)

A

Autosomal Recessive (AR)

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32
Q

Group together due to their certain similarities, one of which is the presence of SMALL PLATELETS in the peripheral smear.

A

TORCH INFECTIONS

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33
Q

TORCH stands for

A

● TOXOPLASMA
● OTHER AGENTS
● RUBELLA
● CYTOMEGALOVIRUS
● HERPESVIRUS

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34
Q

What is the letter T in TORCH infections?

A

○ TOXOPLASMA

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35
Q

● Presence of Giant platelets
● Thrombocytopenia

A

BERNARD - SOULIER SYNDROME (BSS)

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36
Q

BERNARD - SOULIER SYNDROME (BSS)

Receptor that combine with
your platelets

A

Gp1b/IXb/V complex deficiency

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37
Q

Genetic inheritance of GRAY PLATELET SYNDROME (GPS)

A

Autosomal Recessive (AR)

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38
Q

GRAY PLATELET SYNDROME (GPS)

One of the storage pool defects

A

Alpha Granules deficiency

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39
Q

● Presence of Large Gray platelets
● Mild bleeding tendencies
● Thrombocytopenia
● Fibrosis of the marrow

A

GRAY PLATELET SYNDROME (GPS)

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40
Q

Genetic inheritance of MAY - HEGGLIN ANOMALY (MHA)

A

Autosomal Dominant

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41
Q

MYH9 MUTATIONS

Kinds
All presents
Large platelets

(4)

A

● Fechtner syndrome
● Sebastian syndrome
● Epstein syndrome
● MHA (May - Hegglin Anomaly)

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42
Q

● Variable thrombocytopenia
● Presence of spindle shaped
inclusions in WBC’s
○ Döhle-body like inclusions

A

MAY - HEGGLIN ANOMALY (MHA)

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43
Q

All of the following exhibits large platelets except:

a. Flechtner syndrome
b. Epstein syndrome
c. GPS
d. WAS

A

d. WAS

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43
Q

● Also a WBC disorder
● Presence of Döhle body like
inclusions
● Presence of Giant platelets
● Leukopenia
○ Low WBC count

A

MAY - HEGGLIN ANOMALY (MHA)

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44
Q

SHAPE OF PLATELETS

● Shape varies/state whether active or resting

● Bicon_______ - Red Blood Cell
● Bicon_______ - Platelets

A

● Biconcave - Red Blood Cell
● Biconvex - Platelets

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44
Q

All of the following are characterized by small platelets except:

a. WAS
b. Cytomegalovirus infection
c. Herpesvirus infection
d. BSS

A

d. BSS

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45
Q

Biconvex shape or
disk-shaped (small plates)

A

Resting and Circulating
platelets

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46
Q

Spherical with pseudopods
(Projections)

A

Activated platelets

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47
Q

CYTOPLASM OF PLATELETS

On a Wright Stained PBS, platelets appear

A

Lavender and Granular

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48
Q

● Chromomere and Hyalomere are two major parts of the cytoplasm

A

Light Microscope

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49
Q

Platelets are only 2-4 um

A

Light Microscope

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50
Q

● Centrally-located
● Granular

A

Chromomere (aka. Granulomere)

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50
Q

To see other minute structures of platelets like cytoskeleton and
dense tubular system

A

Electron Microscope

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51
Q

aka. Granulomere

A

Chromomere

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52
Q

● Peripherally located
● Non-granular

A

Hyalomere

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53
Q

● Size: 30-50 um
● Has a multilobated nucleus and
abundant granular cytoplasm

A

MEGAKARYOCYTES

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54
Q

Aka. Megakaryopoiesis

A

MEGAKARYOCYTOPOIESIS

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55
Q

● Process by which megakaryocytes develop in the bone marrow
● Platelets are products of Megakaryocytes

A

MEGAKARYOCYTOPOIESIS

56
Q

Largest Cells in bone marrow

A

MEGAKARYOCYTES

57
Q

● Less than 0.5% of all bone marrow
● On normal bone marrow smear: 2 to 4 per 10x LPF (LPO)

A

MEGAKARYOCYTES

58
Q

In a normal bone marrow smear, how many megakaryocytes are seen?

A. 2-4 megakaryocytes/100x field
B. 2-4 megakaryocytes/10x field

A

B. 2-4 megakaryocytes/10x field

59
Q

● Hormone that acts a platelet
production
● Major regulator of platelet production

A

THROMBOPOIETIN (TPO)

60
Q

THROMBOPOIETIN (TPO) is primarily produced by the ________

A

Liver

60
Q

● Possesses 23% homology with
erythropoietin (EPO)

A

THROMBOPOIETIN (TPO)

61
Q

THROMBOPOIETIN (TPO) weight (w/unit)

A

70,000 dalton molecule

62
Q

Coagulation factor also
produced by the _____

A

Liver

63
Q

Concentration in plasma

● Thrombopoietin concentration in the plasma is ___________ proportional to platelet and megakaryocyte mass, suggesting that membrane binding and consequent removal of TPO by
thrombocytes is the primary platelet count control mechanism

A

● Thrombopoietin concentration in the plasma is inversely proportional to platelet and megakaryocyte mass, suggesting that membrane binding and consequent removal of TPO by
thrombocytes is the primary platelet count control mechanism

64
Q

● Stimulates megakaryocytopoiesis (Some of the cytokines that function with it to stimulate this process include IL-3, IL-6, AND IL-11)

A

THROMBOPOIETIN (TPO)

65
Q

● Induces the proliferation and
maturation of megakaryocytes
● Induces thrombocytopoiesis

A

THROMBOPOIETIN (TPO)

66
Q

○ TPO receptor site present at all
maturation stages
○ From BRU-Meg to PLTs

A

MPL - myeloproliferative leukemia virus

67
Q

How many is the megakaryocyte lineage-committed progenitor stage

A

3

68
Q

● Least Mature
● Participate in normal mitosis

A

Burst-forming unit (BFU-Meg)

69
Q

● Participate in normal mitosis

A

Colony-forming unit (CFU-Meg)

70
Q

● Most Mature
● Loses its capacity to divide through normal mitosis
● Perform Endomitosis

A

Light-density CFU (LD-CFU-Meg)

71
Q

All of these progenitors resemble:

A

Lymphocytes

72
Q

○ Process involving nuclear and cytoplasmic division.
○ It divides forming 2 daughter cells
○ BFU-Meg and CFU-Meg

A

Normal Mitosis

72
Q

○ Nuclear division without cytoplasmic division
○ LD-CFU-Meg

A

Endomitosis

73
Q

Terminal Megakaryocyte Differentiation Stages

A

stages wherein the observers are able to recognize the unique Wright-stained morphology of the cells in bone marrow smears or H and E - stained bone marrow biopsy sections

74
Q

● least differentiated
● cannot be reliably distinguished
from myeloblasts or pronormoblasts (light microscopy)

A

MK-I STAGE aka Megakaryoblast

75
Q

● identified by the appearance of
nuclear lobularity

A

MK-II STAGE aka Promegakaryocyte

75
Q

● begins to develop MOST of its
cytoplasmic ultrastructure (including α-granules, dense granules, and the demarcation
system (DMS))

A

MK-I STAGE aka Megakaryoblast

76
Q

● most abundant
● easily recognized at 10X magnification
○ (basis is size: 30 to 160 μm)

A

MK-III STAGE
aka Megakaryocyte

77
Q

● largest cell in the bone marrow
● At the full maturation of the cell, platelet shedding proceeds.

A

MK-III STAGE
aka Megakaryocyte

78
Q

Which of the following performs platelet shedding?

a. Megakaryoblast
b. Promegakaryocyte
c. Immature megakaryocyte
d. Mature megakaryocyte

A

d. Mature megakaryocyte

78
Q

● One megakaryocyte may shed
________-________ platelets.

A

2000-4000 platelets

79
Q

● One megakaryocyte may shed
2000-4000 platelets.

A

MK-III STAGE
aka Megakaryocyte

79
Q

Platelet shedding – aka:

A

Thrombocytopoiesis or Thrombopoiesis

○ aka Platelet Production

80
Q

● ⅔ platelets from the bone marrow travels to the blood
● ⅓ platelets goes to the spleen

A

MK-III STAGE aka Megakaryocyte

81
Q

According to Rodaks, at full maturity of the cell, a single megakaryocyte sheds about ____ to _______ platelets all in all

A

2000 to 4000

82
Q

Nucleus

MK-I MK-II MK-III

A

Round - Indented - Multilobed

83
Q

Nucleoli

MK-I MK-II MK-III

A

2 to 6 - Variable - Not visible

84
Q

Chromatin

MK-I MK-II MK-III

A

Homogenous

Moderately condensed

Deeply and variably condensed

85
Q

N:C ratio

MK-I MK-II MK-III

A

3:1 - 1:2 - 1:4

86
Q

Mitosis

MK-I MK-II MK-III

A

Absent - Absent - Absent

87
Q

Endomitosis

MK-I MK-II MK-III

A

Present - Ends - Absent

88
Q

Cytoplasm

MK-I MK-II MK-III

A

Basophilic

Basophilic and granular

Azurophilic and granular

89
Q

Demarcation System

MK-I MK-II MK-III

A

Present - Present - Present

90
Q

Of the three, which are the precursors?

A

MK-I, MK-II

91
Q

○ ultimately delineates individual platelets during thrombocytopoiesis
○ Though present in all megakaryocyte precursors, only one performs thrombopoiesis which is MK-III

A

Demarcation System

92
Q

Hematopoietic cells capable of endomitosis

A

LD-CFU-MEG, MK-I, MK-II

93
Q

The megakaryocyte progenitor that undergoes endomitosis is?

a. MK-I (Precursor)
b. BFU-MEG
c. CFU-MEG
d. LD-CFU-MEG

A

d. LD-CFU-MEG

94
Q

studied using scanning and transmission electron microscopy, flow cytometry, and molecular sequencing

A

PLATELET ULTRASTRUCTURE

95
Q

● selectively permeable
● provides phospholipids that support platelet activation internally and plasma coagulation externally

A

PLATELET PLASMA MEMBRANE

96
Q

● anchored within the membrane are glycoproteins and proteoglycans

A

PLATELET PLASMA MEMBRANE

97
Q

found in the outer, plasma layer

A

Neutral phospholipids

97
Q

● phosphatidylcholine and
● sphingomyelin

A

Neutral phospholipids

98
Q

support platelet activation by supplying arachidonic acid

A

phosphatidylinositol

98
Q

found in the inner, cytoplasmic layer

A

Anionic or polar
phospholipids

99
Q

(3) Found in the inner, cytoplasmic layer

A

● phosphatidylinositol
● phosphatidylethanolamine
● phosphatidylserine

100
Q

flips to the outer surface upon
activation and is the charged phospholipid surface on which 2
coagulation pathway complexes assemble

A

phosphatidylserine

101
Q
A
101
Q
A
102
Q

The 2 complexes are the:

A

TENASE complex and
PROTHROMBINASE complex

102
Q

● the platelet membrane surface
● cellular ultrastructural component unique to the platelet

A

GLYCOCALYX

103
Q

● absorbs albumin, fibrinogen, and other plasma proteins through endocytosis

A

GLYCOCALYX

103
Q

A.k.a GP RECEPTORS

A

GLYCOPROTEIN PLATELET MEMBRANE RECEPTORS

103
Q

Four (4) Kinds of GP Receptors:

A

○ GP Ia/IIa (1a, 2a)
○ GP VI (6)
○ GP Ib/IX/V (1b, 9, 5)
○ GP IIb/IIIa (2b, 3a)

104
Q

vWF specific site

A

GP Iba

104
Q

Bernard Soulier Syndrome

Thrombin cleaves a site on

A

GP V (5)

105
Q
  • Large platelets
  • Autosomal Recessive
  • GPIb/IX/V complex
A

Bernard Soulier Syndrome (BSS)

106
Q

GP IIb/IIIa

A

Glanzmann’s thrombasthenia

107
Q

A molecule that binds to another molecule

A

Ligand

108
Q

○ Used especially to refer to a small molecule that specifically binds to a larger molecule
○ Molecules that bind to the receptor

A

Ligand

108
Q

CAM means

A

Cell Adhesion Molecule

109
Q

STR means

A

Seven- Transmembrane Repeat

110
Q

Activation of Platelets

Thrombin

A

PAR 1
PAR 4

111
Q

Activation of Platelets

Adenosine diphosphate (ADP)

A

P2Y1
P2Y12

112
Q

Activation of Platelets

Thromboxane A2 (TXA2):

A

TPα and TPβ

113
Q

Activation of Platelets

Epinephrine (Adrenaline)

A

α2-adrenergic

114
Q

A product of eicosanoids pathway of the platelets
● Activates the platelets

A

Thromboxane A2 (TXA2)

115
Q

Also called as prostacyclin

A

Prostaglandin I2 (PGI2)

115
Q

● A product of eicosanoids pathway of the blood vessels
● Inhibitor of platelet aggregation
● Strong vasodilator

A

Prostaglandin I2 (PGI2)

116
Q

● A low-affinity receptor for the immunoglobulin Fc portion (has a role in a perilous condition known as heparin-induced
thrombocytopenia [HIT])

A

FcγIIA (CD32)

116
Q

Part of the platelets that receive ligand

A

Receptor

116
Q

has a role in a perilous condition
known as

A

heparin-induced thrombocytopenia [HIT]

116
Q

Inhibitor of Platelets

Prostaglandin I2 (PGI2)

A

IP

116
Q

● During the platelet shape change, it also reassembles in long parallel bundles to provide rigidity to the pseudopods.
○ It must be firm/hard to prevent
the platelets from breaking apart.

A

MICROTUBULES

116
Q

quantification by flow cytometry is a means for measuring in vivo platelet activation

A

P-selectin (CD 62)

117
Q

● An integrin that helps platelet binding to endothelial cells, WBCs, and one another

A

P-selectin (CD 62)

117
Q

Control platelet shape change, extension of pseudopods and section of granule contents

A

PLATELET CYTOSKELETON

117
Q

● Found on the α-granule membranes of the resting platelet but travels via the SCCS to the surface of activated platelets

A

P-selectin (CD 62)

118
Q

● Thick, circumferential bundles of these maintains platelet’s discoid shape
○ Helps the platelets to become
biconvex in shape.
● Reside just within, although NOT touching, the plasma membrane

A

MICROTUBULES

118
Q

Microtubules, actin microfilaments, and intermediate
filaments

A

PLATELET CYTOSKELETON

118
Q

Formed by Tubulins

A

MICROTUBULES

119
Q

● Platelets become round when
microtubules disassemble in the cold. Upon warming to 37°C, they recover their original discoid shape
● It also moves inward on activation to enable expression of alpha granule contents.

A

MICROTUBULES

119
Q

Proteins that form from the
microtubules

A

Tubulins

119
Q

Location of MICROTUBULES

A

Plasma Membrane