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Coagulation > Exam 3 > Flashcards

Exam 3 Flashcards

(110 cards)

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1
Q

abnormal clot in vitro, retraction and normal plt count

A

Glanzmann Thrombasthenia

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2
Q

What is clot retraction

A

volume of a formed clot is reduced through contraction of cytoskeleton of activated plts within the clot

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3
Q

Deficiency or abnormality of the plt membrane GP IIb/ IIIa complex

A

Glanzmann Thrombasthenia

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4
Q

Petechiae, purpura, menorrhagia, GI bleeding, hematuria

A

Glanzmann Thrombasthenia

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5
Q

What levels of GP in Glanzmann thrombasthenia

A

homo severely decreased to no GP IIb/ IIIa
hetero- 50-60% of normal GP IIb/ IIIa

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6
Q

Lab test in Glantzmann Thrombasthenia

A

plts normal
lack of aggregation in response to all plt activating agents
diminished plt activity

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7
Q

How is GT treated

A

normal plt transfusion

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8
Q

What type of platelet disorder is GT

A

platelet aggregation

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9
Q

platelet disorder where GP Ib/ IX/ V complex is missing from platelet surface

A

Bernard Soulier Giant platelet syndrome

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10
Q

If there is an inability to bind to VWF, then platelets have an inability to

A

adhere to exposed subendothelial cells, causes bleeding

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11
Q

In childhood: Ecchymoses, epistaxis, gingival bleeding

A

Bernard-Soulier Giant platelet syndrome

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12
Q

How is Bernard Soulier treated

A

cannot be correct by adding plasma
afibrinolytic therapy- treat the mucosal bleeding

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13
Q

What glycoprotein defect is most often the cause of BSS

A

GP Ib alpha

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14
Q

normal ADP, epi, collagen, arachadonic
no response to ristocetin

A

What lab results can be expected from BSS

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15
Q

What is the main disorder of plt aggregation
and plt adhesion?

A

aggregation- Glanzmann Thrombsthenia
adhesion- Bernard Soulier Giant plt

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16
Q

normal plt count
mucocutaneous hemorrhage, hematuria, epistaxis, easy bruising

A

platelet secretion

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17
Q

What are dense granules

A

storage site for serotonin, nucelotides, Ca and pyrophosphate

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18
Q

Deficiency associated with albinism

A

Dense granules

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19
Q

Plt aggregation test shows an aggregation pattern affected by ADP
What deficiency is this

Arachidonic acid …
epi and low dose ADP …..
Collagen …..

A

dense granules
Arachidonic acid … no response
epi and low dose ADP …..primary wave, no secondary
Collagen ….. decreased

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20
Q

dense granule deficiency that shows defective lysosomal function

A

Hermansky-Pudlak syndrome

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21
Q

Swiss cheese plts

A

Hermansky-Pudlak syndrome

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22
Q

Dense granule deficiency that causes giant lysosomal granules

A

Chediak Higashi syndrome

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23
Q

Dense granule deficiency that causes microthrombocytopenia

A

Wiskottp-Aldrich Syndrome WAS

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24
Q

Dense granule deficiency that is autosomal recessive and causes an absence of radial bones

A

Thrombocytopenia with absent Radii syndrome
TAR

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25
What are alpha granules
storage site for proteins produced by the megakaryocyte or present in plasma and taken up by plts
26
Primary reason for platelets to have a granular appearance
alpha granules
27
absence of alpha granules
gray platelet syndrome
28
large plts with a gray appearance
gray plt syndrome
29
Which part of the platelet is affect in storage pool diseases
dense granules
30
Deficiency that leads to lifelong mild bleeding disorder, platelets do not adhere to collagen
collagen receptor deficiencies
31
What are the 3 ADP receptors
P2X1 - Ca ion influx P2Y1 Ca mobilization and shape change in response to ADP P2Y12 macroscopic plt aggregation
32
No Phospholipid flip
Scott syndrome no binding of vitamin K dependent factors
33
platelets always in activated state
Stormorken syndrome
34
What can case acquired defects of platelet function
drug ingestion
35
What drug irreversible acetylates/ inactivates cyclooxygenase
aspirin
36
What is not affected by aspirin
endothelial cells- produce nitric oxide, a plt inhibitor, making them unaffected by aspirin
37
drugs that inhibit cyclooxygenase are ____
reversible
38
How do P2Y12 ADP receptor inhibitors work
plt activation and aggregation are induced by ADP and inhibited
39
Clopidogrel, prasugrel, ticlopidine, ticagrelor, cangrelor
P2Y12 ADP receptor inhibitors
40
How does clopidogrel (plavix) work
needs to be converted to an active drug by a liver enzyme
41
CYP2C19 gene
clopidogrel plavix
42
Platelet drug that interferes with fibrinogen, inhibits plt aggregation
GP IIb/ IIIa receptor inhibitor
43
Drug used with patients under percutaneous coronary intervention
GP IIb/ IIIa receptor inhibitors
44
Drug that inhibits thrombin by blocking binding site on PAR-1
PAR-1 antagonist
45
P2Y12 inhibitors when taken with aspirin inhibit plt function
46
platelet disorder that shows PV, CML, ET, CMF also decreased aggregation and secretion in response to epi, ADP and collagen
MPNs myeloproliferative neoplasms
47
plt disfunction that arises from coating of plt membrane by paraprotein
multiple myeloma and waldenstrom macroglobulinemia
48
causes plt activation and fragmentation in the extracorporeal circuit
CPB cardiopulmonary bypass surgery
49
associated with hemostatic abnormalities alcoholic cirrhosis
liver disease
50
rare disorder where there is an absence or near absence of fibrinogen, causing abnormal platelet function
hereditary afrinogenemia
51
platelets aggregate at lower concentrations of aggregating agents than normal
hyperaggregable plts
52
sticky plt syndrome
hyperaggregable plts
53
Hereditary Vascular disorder where thin walled blood vessels with a discontinuous endothelium
Hereditary Hemorrhagic Telangiectasia Rendu Osler Weber Syndrome
54
giant cavernous hemangiomas (vascular tumors), present at ___
Hemangioma Thrombocytopenia Kasabach Merrit Syndrome present at birth
55
hyperextensible skin, hypermobile joints, defects in collagen production
Ehlers-Danlos Syndrome
56
allergy associated with foods, drugs, cold, insect bites, vaccinations
Henoch Schonlein purpura allergic purpura
57
proteins coat the plt membrane- causes abnormalities in plt aggregation, adhesion and actibation
paraproteinemia
58
Deposition of abnormal quantities of amyloid protein in tissues
amyloidosis
59
fibrous protein consisting of rigid, aggregated fibrils
amyloid
60
dark flat blotches, leave ae spots, due to lack of collagen support
Senile pupura
61
when antibodies develop to vessel wall components and create complexes that change the wall permeability
Drug induced vascular purpuras
62
What is epistaxis
nose bleeds
63
small pinpoint hemorrhages, 1 mm diameter
Petechiae
64
3mm diameter, generally round
purpura
65
1cm or larger, irregular in shape
eccchymoses
66
Large plts- megathrombocytes dohle like bodies in neutrophils mostly asymptomatic normal plt function
May-Hegglin Anomaly
67
What is the cause of most congenital types of plt disorders
impaired plt PRODUCTION due to chromosomal abnormalities or genetic defects BM does not have enough megakaryocytes
68
What is the normal plt reference range
150-450
69
What is the range for thrombocytopenia
<100
70
What is the most common cause of clinically important bleeding?
thrombocytopenia
71
severe neonatal thrombocytopenia that causes absence or extreme hypoplasia of radial bones
TAR syndrome
72
BM failure causes no megakaryocytes to be produced, TPO receptor is not functioning can develop into luekemia, aplastic anemia, myelodysplasia petechiae and bleeding in infants at birth
Congenital Amegakaryocytic thrombocytopenia
73
thrombocytopenia due to incomplete megakaryocyte differentiation normal plt functon and morphology absent or mild bleeding
autosomal dominant thrombocytopeniaa
74
Disorders due to decreased plt production
X-linked thrombocytopenia May-Hegglin Anomaly TAR syndrome Congenital Amegaakaryocytic Thrombocytopenia Autosomal dominant thrombocytopenia
75
What can cause neonatal thrombocytopenia (platelet production issue)
TORCH syndrome infection - toxoplasmosis, rubella, CMV (most common), herpes maternal ingestion of sulfonamides Impaired plt production Increased plt consumption or sequestration
76
What are 3 disorders that lead to decreased plt production
infections, chronic alcoholism, drug induced
77
Disorders that cause Immune platelet destruction
ITP Immuno drug induced thrombocytopenia neonatal immune mediated thrombocytopenia posttransfusion Purpura secondary thrombocytopenia, immune mediated
78
What is ITP
immune thrombocytopenia purpura
79
Distinguish ITP acute chronic
acute- kids, vaccines, infections chronic- adults, mostly F
80
onset bruising, petechiae and epistaxis on normally healthy child, they had just received a vaccine
Acute ITP
81
What causes chronic ITP
IgG antibodies made against plts,
82
What is menorrhagia and what condition can it be seen in
very heavy period, chronic ITP
83
What lab test to expect with ITP
plt count low plt morpho normla BM hyperplasia coag- abnormal if plts are non functioning
84
What type of Drug induced thrombocytopenia immune complex induced, Drug induced autoabs, hapten induced abs, drug dependet abs abs interact with plts only in presence of drug haptens act as a complete antigen no drug presence needed heparin induced
dependent- abs interact with plts only in presence of drug hapten induced-haptens act as a complete antigen drug induced- no drug presence needed immune complex induced-heparin induced
85
What is neonatal alloimmune thrombocytopenia NAIT
IgG from mom crosses placenta and cause thrombocytopenia in fetus
86
What is neonatal autoimmune thrombocytopenia
passive transfer of maternal ITP autoantibodies
87
mostly patients that are multiparous, middle aged women, usually have history of a blood transfusion 1 week after transfusion shows purpura
PTP post transfusion purpura
88
What causes PTP
alloantibodies to antigens on plts in transfused product
89
thrombocytopenia due to loss of ADAMTS13
TTP thromotic thrombocytopenic purpura Mochcowitz syndrome
90
What does ADAMTS13 do
cuts VWF into small pieces allowing plts to be triggered
91
Who is more commonly affected by TTP
women 30-40
92
What lab tests to expect with TTP
intravascular hemolysis BM- increased megakaryocytes schistocytes normal coag tests
93
How to treat TTP
infusion of plasma that are deficient of ADAMTS13 UL-VWF multimers removed
94
What is HUS
Hemolytic Uremic syndrome thrombotic microangiopathies caused by bacteria mostly Shigella in kids or E. coli O157 cause damage to colon- bloody diarrhea
95
What can cause adult HUS
immunosuppressive drugs or chemo, damaged kidneys, usually will need dialysis
96
What lab results to expect with HUS
hemolytic anemia renal failure thrombocytopenia
97
which is more severe HUS or TTP in terms of thrombocytopenia in terms of RBC anemia
TTP both
98
What is DIC
coag cascade activated and traps plts in fibrin clot
99
DIC and TTP which is red and which is white clots
DIC-Red TTP-White
100
What causes a big spleen syndrome
increased level of plts make spleen sequester more, there are too many plts produced because the plt count is low in blood vessels, BM is making more to compensate
101
What is the range for thrombocytosis
more than 450
102
What is reactive thrombocytosis
increased plt count due to inflammation trauma or underlying condition
103
What is postsplenectomy thrombocytosis
spleen is removed causes high plt count
104
What is Kawasaki disease
disorder caused by inflammation of the walls of small arteries in body
105
Who is most affected by Kawasaki disease
infants and young kids of Japanese descent
106
What lab tests to expect with exercise induced thrombocytosis
MPDs extreme values
107
What is the most common cause of thrombocytosis
Essential thrombocythemia ET
108
What lab results to expect in ET
way too many megakaryocytes in BM super high plt count abnormal plts, clumped agranular
109
Who is most affected by ET
middle aged/ older patients
110
Digital pain, gangrene, erythromelalgia, hemorrhagic episodes
ET

Coagulation (7 decks)

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