2.2 Platelet and Vascular Disorders Flashcards

1
Q

Thrombotic thrombocytopenic purpura (TTP) is characterized by:
A. Prolonged PT
B. Increased PLT aggregation
C. Thrombocytosis
D. Prolonged APTT

A

B. Increased PLT aggregation

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2
Q

Thrombocytopenia may be associated with:
A. Splenectomy
B. Hypersplenism
C. Acute blood loss
D. Increased proliferation of pluripotent stem cells

A

B. Hypersplenism

Hypersplenism is associated with thrombocytopenia. In this condition, up to 90% of PLTs can be sequestered in the spleen, causing decreases in circulatory PLTs. Splenectomy, acute blood loss, and increased proliferation of pluripotent stem cells are associated with thrombocytosis.

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3
Q

Aspirin prevents PLT aggregation by inhibiting the action of which enzyme?
A. Phospholipase
B. Cyclo-oxygenase
C. Thromboxane A2 (TXA2) synthetase
D. Prostacyclin synthetase

A

B. Cyclo-oxygenase

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4
Q

Normal PLT adhesion depends on:
A. Fibrinogen
B. Glycoprotein Ib
C. Glycoprotein IIb-IIIa complex
D. Calcium

A

B. Glycoprotein Ib

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5
Q

Which of the following test results is normal in a patient with classic von Willebrand disease?
A. PLT aggregation
B. APTT
C. PLT count
D. Factor VIII:C and von Willebrand factor (VWF) levels

A

C. PLT count

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6
Q

Bernard-Soulier syndrome is associated with:
A. Decreased factor IX
B. Decreased factor VIII
C. Thrombocytopenia and giant PLTs
D. Abnormal PLT function test results

A

C. Thrombocytopenia and giant PLTs

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7
Q

When performing PLT aggregation studies, which set of PLT aggregation results would most likely be associated with Bernard-Soulier syndrome?
A. Normal PLT aggregation to collagen, adenosine diphosphate (ADP), and ristocetin
B. Normal PLT aggregation to collagen, ADP, and epinephrine (EPI); decreased aggregation to ristocetin
C. Normal PLT aggregation to EPI and ristocetin; decreased aggregation to collagen and ADP
D. Normal PLT aggregation to EPI, ristocetin, and collagen; decreased aggregation to ADP

A

B. Normal PLT aggregation to collagen, ADP, and epinephrine (EPI); decreased aggregation to ristocetin

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8
Q

Which set of PLT responses would be most likely associated with Glanzmann thrombasthenia?
A. Normal PLT aggregation to ADP and ristocetin; decreased aggregation to collagen
B. Normal PLT aggregation to collagen; decreased aggregation to ADP and ristocetin
C. Normal PLT aggregation to ristocetin; decreased aggregation to collagen, ADP, and EPT
D. Normal PLT aggregation to ADP; decreased aggregation to collagen and ristocetin

A

C. Normal PLT aggregation to ristocetin; decreased aggregation to collagen, ADP, and EPT

Glanzmann thrombasthenia is a disorder of PLT aggregation. PLT aggregation is normal in response to ristocetin, but abnormal in response to collagen, ADP, and EPI.

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9
Q

Which of the following is a characteristic of acute immune thrombocytopenic purpura?
A. Spontaneous remission within a few weeks
B. Predominately seen in adults
C. Nonimmune PLT destruction
D. Insidious onset

A

A. Spontaneous remission within a few weeks
##Footnote
Acute immune thrombocytopenic purpura is an immune-mediated disorder found predominantly in children. It is commonly associated with infection (primarily viral). It is characterized by abrupt onset, and spontaneous remission usually occurs within several weeks.

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10
Q

TTP differs from DIC in that:
A. APTT is normal in TTP but prolonged in DIC
B. Schistocytes are not present in TTP but are present in DIC
C. PLT count is decreased in TTP but normal in DIC
D. PT is prolonged in TTP but decreased in DIC

A

A. APTT is normal in TTP but prolonged in DIC

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11
Q

Several hours after birth, a baby boy develops petechiae and purpura and hemorrhagic diathesis. The PLT count is 18 x 10^9/L. What is the most likely explanation for the low PLT count?
A. Drug-induced thrombocytopenia
B. Secondary thrombocytopenia
C. Neonatal alloimmune thrombocytopenia
D. Neonatal DIC

A

C. Neonatal alloimmune thrombocytopenia

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12
Q

Which of the following is associated with post-transfusion purpura (PTP)?
A. Nonimmune thrombocytopenia/alloantibodies
B. Immune-mediated thrombocytopenia/alloantibodies
C. Immune-mediated thrombocytopenia/autoantibodies
D. Nonimmune-mediated thrombocytopenia/autoantibodies

A

B. Immune-mediated thrombocytopenia/alloantibodies

PTP is a rare form of alloimmune thrombocytopenia characterized by severe thrombocytopenia occurring after transfusion of blood or blood products. PTP is caused by antibody-related PLT destruction in previously immunized patients. In the majority of cases, the alloantibody produced is against P1A1(HPA-1a).

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13
Q

Hemolytic uremic syndrome (HUS) is associated with:
A. Fever, thrombocytosis, anemia, and renal failure
B. Fever, granulocytosis, and thrombocytosis
C. Escherichia coli 0157:H7
D. Leukocytosis and thrombocytosis

A

C. Escherichia coli 0157:H7

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14
Q

Storage pool deficiencies are defects of:
A. PLT adhesion
B. PLT aggregation
C. PLT granules
D. PLT production

A

C. PLT granules

Storage pool deficiencies are defects of PLT granules. Most commonly, a decreased in PLT-dense granules is present with decreased release of ADP, ATP, calcium, and serotonin from PLT-dense granules.

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15
Q

Lumi-aggregation measures:
A. PLT aggregation only
B. PLT aggregation and adenosine triphosphate (ATP) release
C. PLT adhesion
D. PLT glycoprotein Ib

A

B. PLT aggregation and adenosine triphosphate (ATP) release

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16
Q

Neurological findings may be commonly associated with which of the following disorders?
A. HUS
B. TTP
C. ITP
D. PTT

A

B. TTP

TTP is characterized by neurological problems, fever, thrombocytopenia, MAHA, and renal failure.

17
Q

Which of the following is correct regarding acquired TTP?
A. Autoimmune disease
B. Decreased VWF
C. Decreased PLT aggregation
D. Decreased PLT adhesion

A

A. Autoimmune disease

Acquired TTP is an autoimmune disease associated with autoantibodies produced against VWF cleaving enzyme (ADAMTS-13). This deficiency results in an increase in plasma VWF and consequently increased PLT aggregation and thrombocytopenia

18
Q

Hereditary hemorrhagic telangiectasia is a disorder of:
A. PLTs
B. Clotting proteins
C. Fibrinolysis
D. Connective tissue

A

D. Connective tissue

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is a connective tissue disorder associated with telangiectases (dilated capillaries) of the mucous membranes and skin. Lesions may develop on the tongue, lips, palate, face, hands, and nasal mucosa and throughout the gastrointestinal tract. This disorder is an autosomal dominant condition that usually manifests in adolescence or early adulthood.

19
Q

Which of the following prevents PLT aggregation?
A. TXA2
B. Thromboxane B2
C. Prostacyclin
D. Antithrombin (AT)

A

C. Prostacyclin

Prostacyclin is released from the endothelium and is an inhibitor of PLT aggregation TXA2 promotes PLT aggregation. Thromboxane B2 is an oxidized form of TXA2 and is excreted in urine. AT is a physiological anticoagulant.

20
Q

Which defect characterizes Gray syndrome?
A. PLT adhesion defect
B. Dense granule defect
C. Alpha granule defect
D. Coagulation defect

A

C. Alpha granule defect

21
Q

The P2Y12 ADP receptor agonist assay may be used to monitor PLT aggregation inhibition to which of the following drugs?
A. Warfarin
B. Heparin
C. Low-molecular-weight heparin (LMWH)
D. Clopidogrel (Plavix)

A

D. Clopidogrel (Plavix)

The VerifyNow P2Y12 test is used to assess a patient’s response to antiplatelet drugs, such as clopidogrel (Plavix) and prasugrel (Effient). These drugs are given orally along with aspirin for prevention of thrombosis or as alternative antiplatelet drugs for patients who cannot tolerate or are not sensitive to aspirin. Clopidogrel and prasugrel prevent PLT aggregation by irreversibly binding to P2Y12, which is a PLT membrane resceptor for ADP. The VerifyNow P2Y12 test is a whole blood test and uses ADP as an aggregating agent to measure the level of PLT aggregation impaired by these medications. The baseline value for PLT aggregation is established. The percent (%) change from baseline aggregation is calculated and reported as % P2Y12 inhibition.

22
Q

Which of the following instruments can be used to evaluate PLT function?
A. PLT aggregometer
B. VerifyNow
C. PFA-100
D. All of the above

A

D. All of the above

23
Q

Which of the following PLT aggregating agents demonstrates a monophasic aggregation curve when used in the optimal concentration?
A. Thrombin
B. Collagen
C. ADP
D. EPI

A

B. Collagen

Collagen is the only commonly used agent that demonstrates a single-wave (monophasic) response preceded by a lag time.