[MCQs] Disorders of Platelets and Vessel Wall- Part 3

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Disorders of Platelets and Vessel Wall- Part 3

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Congratulations - you have completed Disorders of Platelets and Vessel Wall- Part 3. You scored %%SCORE%% out of %%TOTAL%%. Your performance has been rated as %%RATING%%

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Question 1

Which of the following is false about ADAMTS-13 ?

A	ADAMTS refers to “A Disintegrin-like And Metalloprotease with ThromboSpondin type 1 motif”
B	Human ADAMTS-13 gene is on chromosome 9q34
C	ADAMTS-13 molecule depends upon zinc and calcium ions for activity
D	None of the above

Question 2

Findings in TTP include ?

A	Abdominal pain, nausea, vomiting and weakness
B	Seizures and fluctuating focal deficits
C	Thrombocytopenia without leukopenia
D	All of the above

Question 3

Which of the following laboratory finding does not support diagnosis of TTP ?

A	Increased lactate dehydrogenase
B	Increased direct bilirubin
C	Decreased haptoglobin
D	Decreased haptoglobin

Question 3 Explanation:

Laboratory finding that support diagnosis of TTP include increased LDH, increased “indirect bilirubin”, decreased haptoglobin, increased reticulocyte count with negative direct antiglobulin test. PBF shows schistocytes and polychromasia

Question 4

Which of the following is not used in the treatment of TTP ?

A	Platelet concentrates
B	Plasma exchange
C	Fresh-frozen plasma (FFP)
D	Solvent/detergent-treated plasma (SD-FFP)

Question 4 Explanation:

Plasma exchange remains the mainstay of treatment of TTP. Use of platelet concentrates and Desmopressin, or DDAVP in the treatment of TTP is contraindicated as it leads to extensive platelet aggregation in the CNS

Question 5

Immunomodulatory therapies reported to be successful in refractory or relapsing TTP include ?

A	Rituximab
B	Vincristine
C	Cyclophosphamide
D	All of the above

Question 5 Explanation:

Immunomodulatory therapies reported to be successful in refractory or relapsing TTP include rituximab, vincristine, cyclophosphamide, and splenectomy.

Question 6

Hemolytic Uremic Syndrome is a disease of ?

A	Infancy and early childhood
B	Adolescence
C	Adults
D	Elderly

Question 6 Explanation:

Elderly

Question 7

Features of Hemolytic Uremic Syndrome include all except ?

A	Chronic renal failure
B	Microangiopathic hemolytic anemia
C	Thrombocytopenia
D	Frequently preceded by an episode of diarrhea

Question 7 Explanation:

HUS is a syndrome characterized by acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. In most cases is preceded by an episode of diarrhea. Escherichia coli O157:H7 is the most frequent etiologic serotype. Plasma exchange treatment is not appropriate for hemolytic-uremic syndrome.

Question 8

Thrombocytosis is due to deficiency of ?

A	Vitamin B12
B	Folic acid
C	Iron
D	Calcium

Question 8 Explanation:

Thrombocytosis is almost always due to iron deficiency, reactive thrombocytosis (inflammation, cancer, infection) or underlying myeloproliferative process (essential thrombocythemia or PV)

Question 9

In which of the following disorders, there is absence of the platelet GpIIbIIIa receptor ?

A	Glanzmann’s thrombasthenia
B	Bernard Soulier syndrome
C	Hermansky-Pudlak syndrome
D	Heyde’s syndrome

Question 9 Explanation:

Autosomal recessive inherited qualitative disorders of platelet function include Glanzmann’s thrombasthenia (absence of the platelet GpIIbIIIa receptor) and Bernard Soulier syndrome (absence of the platelet GpIb-IX-V receptor). Edward Glanzmann - Swiss physician (1918)

Question 10

Bleeding symptoms or prevention of bleeding in patients in inherited disorders of platelet dysfunction include ?

A	Prestorage leukodepleted platelets transfusion
B	Desmopressin (DDAVP)
C	EACA or tranexamic acid
D	All of the above

Question 11

Acquired platelet dysfunction can occur in ?

A	Uremia
B	High dose penicillins
C	With cardiopulmonary bypass
D	All of the above

Question 12

Acquired platelet dysfunction due to uremia, respond to ?

A	Dialysis
B	DDAVP
C	Conjugated estrogens
D	All of the above

Question 13

Most common inherited bleeding disorder is ?

A	Glanzmann’s thrombasthenia
B	Bernard Soulier syndrome
C	von Willebrand Disease
D	Hemophilia

Question 13 Explanation:

von Willebrand Disease (vWD) is the most common inherited bleeding disorder with prevalence of ~1%. vWD affects both males & females, while hemophilia mainly affects males.

Question 14

Nationality of Dr. Erik von Willebrand was ?

A	French
B	Finnish
C	Spanish
D	Danish

Question 14 Explanation:

In 1925, Dr. Erik von Willebrand of Finland observed a disorder that was due to a disturbed function of thrombocytes and a general lesion of capillary walls and called it “pseudohemophilia”.

Question 15

Normal plasma vWF level is ?

A	4 mg/L
B	6 mg/L
C	8 mg/L
D	10 mg/L

Question 15 Explanation:

The normal plasma vWF level is 10 mg/L

Question 16

Which of the following vWD is not inherited as autosomal dominant trait?

A	Type I
B	Type IIA
C	Type IIB
D	Type III

Question 16 Explanation:

Except type III vWF disease, all forms are inherited as autosomal dominant traits

Question 17

Which of the following vWD type is most common ?

A	Type I
B	Type IIA
C	Type IIB
D	Type III

Question 17 Explanation:

Type I vWF disease is most common. There is mild to moderate decrease in plasma vWF

Question 18

Which of the following vWD type is most severe ?

A	Type I
B	Type IIA
C	Type IIB
D	Type III

Question 18 Explanation:

Type III vWF disease is a very severe form of vWD with no detectable vWF antigen or activity. Patients are usually the offspring of two parents (usually asymptomatic) with mild type I disease.

Question 19

Which of the following is not a functional platelet disorder of adhesion ?

A	von Willebrand’s disease (vWD)
B	Glanzmann’s thrombasthenia
C	Bernard-Soulier syndrome
D	Uremia

Question 19 Explanation:

Functional platelet disorders of adhesion are either inherited (Bernard-Soulier syndrome, von Willebrand’s disease), or acquired (Uremia, acquired vWD)

Question 20

Which of the following is a functional platelet disorder of aggregation ?

A	Glanzmann’s thrombasthenia
B	Bernard-Soulier syndrome
C	Chediak-Higashi syndrome
D	Hermansky-Pudlak syndrome

Question 20 Explanation:

Functional platelet disorders of aggregation are either inherited (Glanzmann’s thrombasthenia, afibrinogenemia) or acquired (fibrin degradation product inhibition, dysproteinemias, drug induced - ticlopidine, Gp IIb/IIIa inhibitors)

Question 21

Which of the following is not a functional platelet disorder of granule release ?

A	Hermansky-Pudlak syndrome
B	Chediak-Higashi syndrome
C	von Willebrand’s disease (vWD)
D	Gray-platelet syndrome

Question 21 Explanation:

Functional platelet disorders of granule release are inherited (Hermansky-Pudlak syndrome, Chediak-Higashi syndrome, isolated dense () granule deficiency, Gray-platelet syndrome or acquired (cardiopulmonary bypass, myeloproliferative disorders, drug induced - aspirin, NSAIDs).

Question 22

Which of the following is a platelet aggregation agonist ?

A	ADP
B	Thrombin
C	Epinephrine
D	All of the above

Question 22 Explanation:

Agonists of platelet aggregation are adenosine diphosphate (ADP), thrombin or epinephrine

Question 23

vWF precursor subunit is synthesized in ?

A	Endothelial cells
B	Platelets
C	Vascular subendothelium
D	All of the above

Question 23 Explanation:

A single large vWF precursor subunit is synthesized in endothelial cells & megakaryocytes, where it is cleaved & assembled into disulfide-linked multimers in plasma, platelets, & subendothelium

Question 24

Which of the following is false in diagnostic pattern of vWD ?

A	Prolonged clotting time
B	Reduced vWF concentration
C	Reduced vWF biologic activity
D	Reduced factor VIII activity

Question 24 Explanation:

Lab. diagnostic findings in vWD are prolonged BT, reduction in plasma vWF level, a parallel reduction in vWF biologic activity by ristocetin cofactor assay and reduced factor VIII activity

Question 25

Which of the following vWD is due to a defect in factor VIII binding site of vWF ?

A	Type I
B	Type II
C	Type IIn
D	Type III

Question 25 Explanation:

Type IIn vWD is due to a defect in factor VIII binding site of vWF

Question 26

Patients of which type of vWD are the best candidates for desmopressin therapy ?

A	Type I
B	Type IIA
C	Type IIB
D	Type III

Question 26 Explanation:

Patients with type I vWD are the best candidates for desmopressin therapy.

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II. See all quizzes of the Disorders of Platelets and Vessel Wall at here:

Disorders of Platelets and Vessel Wall- Part 1 |Disorders of Platelets and Vessel Wall- Part 2 |Disorders of Platelets and Vessel Wall- Part 3 |Disorders of Platelets and Vessel Wall- Part 4

1. Which of the following is false about ADAMTS-13 ?
A. ADAMTS refers to “A Disintegrin-like And Metalloprotease with ThromboSpondin type 1 motif”
B. Human ADAMTS-13 gene is on chromosome 9q34
C. ADAMTS-13 molecule depends upon zinc and calcium ions for activity
D. None of the above

2. Findings in TTP include ?
A. Abdominal pain, nausea, vomiting and weakness
B. Seizures and fluctuating focal deficits
C. Thrombocytopenia without leukopenia
D. All of the above

3. Which of the following laboratory finding does not support diagnosis of TTP ?
A. Increased lactate dehydrogenase
B. Increased direct bilirubin
C. Decreased haptoglobin
D. Increased reticulocyte count

4. Which of the following is not used in the treatment of TTP ?
A. Platelet concentrates
B. Plasma exchange
C. Fresh-frozen plasma (FFP)
D. Solvent/detergent-treated plasma (SD-FFP)

5. Immunomodulatory therapies reported to be successful in refractory or relapsing TTP include ?
A. Rituximab
B. Vincristine
C. Cyclophosphamide
D. All of the above

6. Hemolytic Uremic Syndrome is a disease of ?
A. Infancy and early childhood
B. Adolescence
C. Adults
D. Elderly

7. Features of Hemolytic Uremic Syndrome include all except ?
A. Chronic renal failure
B. Microangiopathic hemolytic anemia
C. Thrombocytopenia
D. Frequently preceded by an episode of diarrhea

8. Thrombocytosis is due to deficiency of ?
A. Vitamin B12
B. Folic acid
C. Iron
D. Calcium

9. In which of the following disorders, there is absence of the platelet GpIIbIIIa receptor ?
A. Glanzmann’s thrombasthenia
B. Bernard Soulier syndrome
C. Hermansky-Pudlak syndrome
D. Heyde’s syndrome

10. Bleeding symptoms or prevention of bleeding in patients in inherited disorders of platelet dysfunction include ?
A. Prestorage leukodepleted platelets transfusion
B. Desmopressin (DDAVP)
C. EACA or tranexamic acid
D. All of the above

11. Acquired platelet dysfunction can occur in ?
A. Uremia
B. High dose penicillins
C. With cardiopulmonary bypass
D. All of the above

12. Acquired platelet dysfunction due to uremia, respond to ?
A. Dialysis
B. DDAVP
C. Conjugated estrogens
D. All of the above

13. Most common inherited bleeding disorder is ?
A. Glanzmann’s thrombasthenia
B. Bernard Soulier syndrome
C. von Willebrand Disease
D. Hemophilia

14. Nationality of Dr. Erik von Willebrand was ?
A. French
B. Finnish
C. Spanish
D. Danish

15. Normal plasma vWF level is ?
A. 4 mg/L
B. 6 mg/L
C. 8 mg/L
D. 10 mg/L

16. Which of the following vWD is not inherited as autosomal dominant trait?
A. Type I
B. Type IIA
C. Type IIB
D. Type III

17. Which of the following vWD type is most common ?
A. Type I
B. Type IIA

C. Type IIB
D. Type III

18. Which of the following vWD type is most severe ?
A. Type I
B. Type IIA
C. Type IIB
D. Type III

19. Which of the following is not a functional platelet disorder of adhesion ?
A. von Willebrand’s disease (vWD)
B. Glanzmann’s thrombasthenia
C. Bernard-Soulier syndrome
D. Uremia

20. Which of the following is a functional platelet disorder of aggregation ?
A. Glanzmann’s thrombasthenia
B. Bernard-Soulier syndrome
C. Chediak-Higashi syndrome
D. Hermansky-Pudlak syndrome

21. Which of the following is not a functional platelet disorder of granule release ?
A. Hermansky-Pudlak syndrome
B. Chediak-Higashi syndrome
C. von Willebrand’s disease (vWD)
D. Gray-platelet syndrome

22. Which of the following is a platelet aggregation agonist ?
A. ADP
B. Thrombin
C. Epinephrine
D. All of the above

23. vWF precursor subunit is synthesized in ?
A. Endothelial cells

B. Platelets
C. Vascular subendothelium
D. All of the above

24. Which of the following is false in diagnostic pattern of vWD ?
A. Prolonged clotting time
B. Reduced vWF concentration
C. Reduced vWF biologic activity
D. Reduced factor VIII activity

25. Which of the following vWD is due to a defect in factor VIII binding site of vWF ?
A. Type I
B. Type II
C. Type IIn
D. Type III

26. Patients of which type of vWD are the best candidates for desmopressin therapy ?
A. Type I
B. Type IIA
C. Type IIB
D. Type III

Disorders of Platelets and Vessel Wall

[MCQs] Disorders of Platelets and Vessel Wall- Part 3

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Disorders of Platelets and Vessel Wall- Part 3

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