Qualitative Platelet

Question 1

Which of the following is a characteristic of Glanzmann Thrombasthenia?
A. Deficiency of GP Ib/IX/V complex
B. Giant platelets
C. Lack of GP IIb/IIIa receptors
D. Absence of alpha granules

Answer 1

C

Question 2

Bernard-Soulier Syndrome is associated with which defect?
A. Platelet secretion
B. Platelet aggregation
C. Platelet adhesion
D. Fibrinogen formation

Answer 2

C

Question 3

What is the mode of inheritance for Wiskott-Aldrich Syndrome?
A. Autosomal dominant
B. Autosomal recessive
C. X-linked recessive
D. Mitochondrial

Answer 3

C

Question 4

Which of the following is not typically found in Hermansky-Pudlak Syndrome?
A. Oculocutaneous albinism
B. Giant lysosomal granules
C. Platelet dense granule deficiency
D. Ceroid deposition

Answer 4

B

Question 5

Von Willebrand Disease Type 3 is characterized by:
A. Partial deficiency of VWF
B. Qualitative defect in VWF
C. Complete deficiency of Factor VIII
D. Deficiency of HMW multimers only

Answer 5

C

Question 6

What is the treatment of choice for severe bleeding in Gray Platelet Syndrome?
A. Desmopressin
B. Aspirin
C. Platelet transfusion
D. Warfarin

Answer 6

C

Question 7

Which disorder is associated with tyrosinase-positive oculocutaneous albinism and dense granule deficiency?
A. Chediak-Higashi Syndrome
B. Hermansky-Pudlak Syndrome
C. Wiskott-Aldrich Syndrome
D. Stormorken Syndrome

Answer 7

B

Question 8

What is the inheritance pattern of Glanzmann thrombasthenia?
A. X-linked dominant
B. Autosomal dominant
C. Autosomal recessive
D. X-linked recessive

Answer 8

C

Question 9

The primary platelet defect in von Willebrand disease involves impaired:
A. Platelet secretion
B. Platelet adhesion
C. Platelet aggregation
D. Platelet granule formation

Answer 9

B

Question 10

Which drug irreversibly inhibits cyclooxygenase in platelets?
A. Ticagrelor
B. Clopidogrel
C. Aspirin
D. Dipyridamole

Answer 10

C

Question 11

What is the classic triad in Wiskott-Aldrich Syndrome?
A. Albinism, infections, thrombocytopenia
B. Eczema, thrombocytopenia, immunodeficiency
C. Bleeding, anemia, hepatomegaly
D. Petechiae, eczema, hepatosplenomegaly

Answer 11

B

Question 12

Which glycoprotein is defective in Bernard-Soulier Syndrome?
A. GP IIb/IIIa
B. GP Ib/IX/V
C. GP VI
D. GP Ia/IIa

Answer 12

B

Question 13

Which inherited platelet disorder is associated with “Swiss cheese” platelet morphology?
A. Scott Syndrome
B. TAR Syndrome
C. Hermansky-Pudlak Syndrome
D. May-Hegglin anomaly

Answer 13

C

Question 14

What is the characteristic platelet morphology in Gray Platelet Syndrome?
A. Giant granules
B. Gray appearance due to lack of alpha granules
C. Swiss cheese appearance
D. Small dense platelets

Answer 14

B

Question 15

Which of the following is a P2Y12 receptor inhibitor?
A. Eptifibatide
B. Vorapaxar
C. Clopidogrel
D. Abciximab

Answer 15

C

Question 16

Scott Syndrome is characterized by a defect in:
A. Platelet aggregation
B. Platelet secretion
C. Membrane phospholipid scrambling
D. Thromboxane A2 synthesis

Answer 16

C

Question 17

Which of the following is an autosomal dominant inherited platelet disorder?
A. Glanzmann thrombasthenia
B. Bernard-Soulier Syndrome
C. Stormorken Syndrome
D. Wiskott-Aldrich Syndrome

Answer 17

C

Question 18

Which syndrome is associated with giant lysosomal granules and recurrent infections?
A. Hermansky-Pudlak Syndrome
B. TAR Syndrome
C. Chediak-Higashi Syndrome
D. Gray Platelet Syndrome

Answer 18

C

Question 19

Which platelet receptor is responsible for binding fibrinogen during aggregation?
A. GP Ib/IX/V
B. GP VI
C. GP IIb/IIIa
D. P2Y12

Answer 19

C

Question 20

In von Willebrand Disease type 2B, there is:
A. Decreased VWF binding to platelets
B. Increased affinity for GP Ib
C. Complete VWF deficiency
D. Absence of HMW multimers

Answer 20

B

Question 21

Which disorder is associated with congenital absence of the radius bone and thrombocytopenia?
A. Wiskott-Aldrich Syndrome
B. TAR Syndrome
C. May-Hegglin Anomaly
D. Scott Syndrome

Answer 21

B

Question 22

Dipyridamole inhibits platelet function by:
A. Blocking GP IIb/IIIa
B. Inhibiting thrombin generation
C. Inhibiting phosphodiesterase
D. Inhibiting COX-1

Answer 22

C

Question 23

A patient with small platelets, eczema, and immunodeficiency most likely has:
A. Bernard-Soulier Syndrome
B. TAR Syndrome
C. Wiskott-Aldrich Syndrome
D. Glanzmann Thrombasthenia

Answer 23

C

Question 24

The platelet function defect in Scott Syndrome involves failure to:
A. Aggregate properly
B. Release alpha granules
C. Flip phospholipids for coagulation factor binding
D. Synthesize thromboxane A2

Answer 24

C

Question 25

The "Swiss cheese" platelet morphology is found in: A. TAR Syndrome B. Hermansky-Pudlak Syndrome C. Gray Platelet Syndrome D. Wiskott-Aldrich Syndrome

Answer 25

B

Question 26

Which drug inhibits platelet aggregation by blocking PAR-1? A. Tirofiban B. Vorapaxar C. Clopidogrel D. Eptifibatide

Answer 26

B

Question 27

Which inherited platelet disorder presents with Dohle-like bodies and thrombocytopenia? A. May-Hegglin Anomaly B. TAR Syndrome C. Scott Syndrome D. Wiskott-Aldrich Syndrome

Answer 27

A

Question 28

What is the primary defect in the thromboxane pathway disorders? A. Impaired ADP receptor signaling B. Decreased fibrinogen binding C. Abnormal arachidonic acid metabolism D. Impaired phospholipid scrambling

Answer 28

C

Question 29

Which platelet granules are primarily deficient in Hermansky-Pudlak Syndrome? A. Alpha granules B. Lysosomal granules C. Dense granules D. All granules

Answer 29

C

Question 30

A patient presents with platelet dysfunction that cannot be corrected with normal plasma. Which condition is most likely? A. von Willebrand Disease B. Bernard-Soulier Syndrome C. Glanzmann Thrombasthenia D. Hemophilia A

Answer 30

B

Question 31

Which of the following disorders involves a deficiency in GP VI, impairing platelet-collagen interaction? A. Bernard-Soulier Syndrome B. Gray Platelet Syndrome C. Scott Syndrome D. GP VI Deficiency

Answer 31

D

Question 32

What is the treatment of choice for mucosal bleeding in Bernard-Soulier Syndrome? A. Desmopressin B. Aspirin C. Platelet transfusion D. Factor VIII concentrate

Answer 32

C

Question 33

Which gene is mutated in Wiskott-Aldrich Syndrome? A. NBEAL2 B. WAS C. ITGA2B D. VWF

Answer 33

B

Question 34

Which syndrome is associated with macrothrombocytopenia and absence of alpha granules? A. Gray Platelet Syndrome B. May-Hegglin Anomaly C. TAR Syndrome D. Stormorken Syndrome

Answer 34

A

Question 35

Which of the following platelet receptor inhibitors is a monoclonal antibody? A. Abciximab B. Tirofiban C. Eptifibatide D. Vorapaxar

Answer 35

A

Question 36

What is a characteristic feature of senile purpura? A. Hemarthrosis B. Blotchy skin lesions on forearms and hands C. Enlarged lymph nodes D. Petechial rash with fever

Answer 36

B