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Coagulation > Exam 3 > Flashcards

Exam 3 Flashcards

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1
Q

What aspect of hemostasis is affected by PLT disorders?

A

Primary hemostasis

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2
Q

What is the major disorder of PLT aggregation?

A

Glanzmann Thrombasthenia

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3
Q

Pathophysiology of Glanzmann-Thrombasthenia

A

a deficiency or abnormality of the PLT membrane GPIIb/GPIIa complex

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4
Q

Signs/Symptoms of Glanzmann Thrombasthenia

A

petechiae, purpura, menorrhagia, GI bleeding, hematuria

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5
Q

What population is Glanzmann Thrombasthenia commonly found in?

A

populations with high levels of inbreeding

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6
Q

What does PLT membrane GPIIb/IIIa bind?

A

fibrinogen, VWF, and fibronectin

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7
Q

What is the aggregation disorder that is associated with abnormal in vitro clot retraction and a normal PLT count?

A

Glanzmann Thrombasthenia

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8
Q

Homozygous or heterozygous conditions more severe?

A

Homozygous

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9
Q

What do the test results look like for Glanzmann Thrombasthenia? (PLT count, PLT morphology, PLT aggregation, PLT procoagulant activity tests)

A

PLT count - normal
PLT morphology - normal
PLT aggregation - lack of aggregation in response to all PLT activating agents
PLT procoagulant activity tests - diminished activity

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10
Q

Treatment of Glanzmann-Thrombasthenia

A

Transfusion of normal PLTs

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11
Q

What is the major disorder of PLT adhesion?

A

Bernard-Soulier (Giant Platelet) Syndrome

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12
Q

What is the pathophysiology of Bernard-Soulier Syndrome?

A

Missing GP Ib/IX/V complex; inability to bind VWF
Causes bleeding

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13
Q

Signs/Symptoms of Bernard-Soulier Syndrome

A

Ecchymoses, epistaxis, and gingival bleeding in childhood

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14
Q

What is the most frequent form of Bernard Soulier Syndrome?

A

Defects in synthesis or expression of GPIb alpha receptor

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15
Q

What will be seen in lab results for Bernard-Soulier Syndrome?

A

No response to ristocetin (ristocetin is an agonist to VWF and those with BSS cannot bind to VWF)

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16
Q

Treatment of Bernard-Soulier Syndrome

A

Afibrinolytic therapy (treat mucosal bleeding)
PLT transfusions (but can lead to developing alloantibodies)

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17
Q

What are Dense Granule Deficiencies caused by?

A

lack of ADP secretion

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18
Q

What deficiency is Hermansky-Pudlak Syndrome? What is it characterized by?

A

Dense granule deficiency; characterized by defective lysosomal function; platelets look like “swiss cheese”

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19
Q

What deficiency causes Chediak-Higashi Syndrome? What is it characterized by?

A

Dense granule deficiency; characterized by giant lysosomal granules

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20
Q

What deficiency causes Wiskott-Aldrich Syndrome? What is it characterized by?

A

Dense granule deficiency; characterized by microthrombocytopenia (microthrombocytes) and severe eczema

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21
Q

What deficiency causes Thrombocytopenia with Absent Radii Syndrome? What is it characterized by?

A

Dense granule deficiency; characterized by absence of radial bones

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22
Q

What deficiency causes Gray Platelet Syndrome? what is it characterized by?

A

alpha granule deficiency; characterized by absence of morphologically recognizable alpha granules in PLTs and large PLTs with a gray appearance

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23
Q

Which part of the platelet is affected in storage pool disorders?

A

Granule deficiencies (alpha and dense)

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24
Q

What is Scott Syndrome?

A

No phospholipid flip occurs; causes severely reduced or no thrombin generation

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25
What is Stormorken Syndrome?
PLTs are always in an activated state; causes mild bleeding tendancy
26
What target does aspirin bind to?
COX-1 in an irreversible form?
27
What target does Clopidogrel, Prasugrel bind to?
ADP P2Y12 irreversibly
28
What target does Ticagrelor and Cangrelor bind to?
ADP P2Y12 reversibly
29
What target does Vorapaxar bind to?
Thrombin PAR-1
30
What target does Abciximab, Eptifibatide, and Tirofiban bind to?
GP IIb/IIIa
31
How does aspirin work as an antiplatelet drug?
Inhibits cyclooxygenase - leads to decreased thromboxane and decreased PLT activation
32
What do P2Y12 (ADP) receptor inhibitors do?
inhibits PLT activation and aggregation induced by ADP
33
What do GPIIb/IIIa receptor inhibitors do?
Interfere with ability to bind FBG
34
What do PAR-1 antagonists do?
inhibits thrombin induced PLT aggregation
35
How does Multiple Myeloma and Waldenstrom Macroglobulinemia affect PLT function?
coating of PLT membrane with paraprotein
36
What is another name for Hereditary Hemorrhagic Telangiectasia?
Rendu-Osler-Weber Syndrome
37
Pathophysiology of Rendu-Osler-Weber Syndrome
thin-walled blood vessels with a discontinuous endothelium and telangiectasias
38
What is another name for Hemangioma-Thrombocytopenia Syndrome? What is it characterized by?
Kasabach-Merritt Syndrome characterized by hemangiomas (vascular tumors)
39
What is Ehlers-Danlos Syndrome?
Defect in collagen production leads to hyperextensible skin, hypermobile joints, bleeding tendencies
40
Acquired vascular disorder: Allergic Purpura - what is another name for it? what is it characterized by?
Henoch-Schonlein Purpura - characterized by allergic purpuras (rashes, IgA mediated)
41
what is paraproteinemia?
Proteins coat the PLT membrane
42
What is senile purpura caused by?
lack of collagen support
43
ecchymoses
bruises
44
menorrhagia
heavy menstrual bleeding
45
epistaxis
nose bleeds
46
petechiae vs purpura vs ecchymoses
petechiae are smaller pinpoint hemorrhages purpura are larger round hemorrhages ecchymoses: bruises that are the largest
47
What PLT count constitutes thrombocytopenia?
< 100 x 10^9 PLTs/L
48
What is the root cause of thrombocytopenic disorders?
Decreased PLT production Increased PLT destruction Abnormal PLT sequestration
49
May-Hegglin Anomaly: decreased PLT production, increased PLT destruction, or abnormal PLT sequestration?
decreased PLT production
50
What can be seen in May-Hegglin Anomaly?
Large PLTs, Dohle-like bodies present in neutrophils, normal PLT function
51
TAR syndrome: decreased PLT production, increased PLT destruction, or abnormal PLT sequestration?
decreased PLT production
52
What can be seen in TAR syndrome?
Severe neonatal thrombocytopenia with absence/extreme hypoplasia of radial bones
53
What are causes of neonatal thrombocytopenia?
TORCH syndrome infections (CMV most common), Maternal ingestion of certain sulfonamides, Impaired PLT production, Increase PLT destruction or sequestration
54
Immune Thrombocytopenic Purpura: decreased PLT production, increased PLT destruction, or abnormal PLT sequestration?
increased PLT destruction
55
Acute Immune Thrombocytopenic purpura (ITP)
Mostly seen in children; preceded by various infections/vaccinations `
56
Chronic Immune Thrombocytopenic purpura (ITP)
Mostly seen in females; autoantibodies to PLTs leading to removal of PLTs from circulation
57
Lab testing for ITP (plt count, plt morphology, bm)
PLT count: low (30-80) PLT morphology: normal BM: megakaryocytic hyperplasia
58
4 mechanisms of drug-induced thrombocytopenia:
1. Drug-dependent antibodies 2. Hapten-induced antibodies 3. Drug-induced autoantibodes 4. Immune complex-induced thrombocytopenia
59
What is the mechanism of Drug-Dependent Antibodies in drug-induced thrombocytopenia?
Antibodies induced by drugs interact with PLTs only in the presence of the drug (example: anti-malarial drugs)
60
What is the mechanism of Hapten-induced antibodies in drug-induced thrombocytopenia?
Drug by itself cannot trigger an immune response, but haptens can act as a complete antigen to which antibodies are made (ex. Penicillin)
61
What is the mechanism of Drug-induced autoantibodies in drug-induced thrombocytopenia?
Drug stimulates the formation of autoantibody and antibody binds to own platelets; when drug goes away, autoantibody still reacts (NO presence of drug required)
62
What is the drug involved in immune complex-induced thrombocytopenia?
Heparin-induced thrombocytopenia (HIT)
63
What is the difference between neonatal alloimmune thrombocytopenia and neonatal autoimmune thrombocytopenia?
Alloimmune: like HDFN for PLTs (mother exposed to fetal antigen she lacks and makes antibodies to that antigen and it crosses placenta) most common cause is HPA-1a antigen Autoimmune: Develops from the passive transfer of material ITP autoantibodies
64
Which population most commonly develops Posttransfusion purpura (PTP)
multiparous women (pregnant multiple times), middle-aged women, almost all with a history of blood transfusion
65
What is another name for TTP? Is it immune or non immune PLT destruction thrombocytopenia?
Moschcowitz Syndrome; non-immune
66
Describe TTP (Moschcowitz Syndrome) (loss of what enzyme activity, signs/symptoms, population affected, lab findings)
loss of ADAMTS13 activity; characterized by MAHA, thrombocytopenia, and neurologic abnormalities. Affects mostly women age 30-40 Lab findings: thrombi composed of PLTs and VWF but very little fibrin or fibrinogen, schistocytosis, intravascular hemolysis, low hemoglobin
67
What is the cause of HUS?
Shigella dysenteriae serotypes (Shiga toxin) E. coli OH serotypes (Shiga-like toxin-1)
68
What is the pathophysiology of HUS?
Toxins attach to renal cells and they lead to renal failure, thrombocytopenia, and MAHA
69
Cardinal signs of HUS
Renal failure Thrombocytopenia Hemolytic anemia (schistocytes, increased retic count)
70
How to differentiate HUS from TTP?
HUS has lack of neurological symptoms and presence of renal dysfunction HUS has lack of other organ involvement HUS thrombocytopenia is not as severe HUS RBC fragmentation and anemia is not as severe
71
Describe DIC
activation of coag cascade results in trapped PLTs in intravascular fibrin clots
72
DIC vs TTP
DIC - thrombi composed of PLTs and FBG (red clots) TTP - thrombi composted of PLTs and VWF (white clots)
73
Is acute DIC or chronic DIC more life threatening?
Acute DIC because there is rapid PLT consumption instead of ongoing, low grade PLT consumption
74
Purpura Fulminans (PF)
associated with acute sepsis (N. meningitidis, Strep pneumo, Group A/B strep)
75
How would a big spleen syndrome affect the PLT count? Why?
Increased levels of PLTs are sequestered in the large spleen so venous blood PLT count is low
76
thrombocytosis PLT count
>450
77
Reactive thrombocytosis
Increased PLT count secondary to inflammation, trauma, or other underlying conditions
78
Postsplenectomy Thrombocytosis
Removal of spleen results in PLT counts >1 MILLION/uL ; expected increase of 30-50% in PLT count for 1-3 months
79
Kawasaki Disease
disorder caused by inflammation of the walls of small and medium sized arteries; affects infants and young children, highest incidence in Japenese people
80
What is the most common cause of thrombocytosis?
Essential Thrombocythemia (ET)
81
What is essential thrombocythemia?
Uncontrolled proliferation of BM megakaryocytes prevalent in middle aged/older patients
82
What are the clinical manifestations of ET?
hemorrhage, PLT dysfunction, thrombosis
83
Clinical findings in ET
Differing sizes of PLTs Clumped PLTs Agranular PLTs Megakaryocyte fragments
84
Which myeloproliferative disease leads to the most extreme thrombocytosis?
Essential Thrombocytopenia

Coagulation (5 decks)

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