[MCQs] Disorders of Platelets and Vessel Wall- Part 4

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Disorders of Platelets and Vessel Wall- Part 4

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Question 1

vWD may be acquired in which of the following situations ?

A	Monoclonal gammopathies of undetermined significance
B	Waldenstrom’s macroglobulinemia
C	Multiple myeloma
D	All of the above

Question 1 Explanation:

Acquired vWD is mostly seen in patients with underlying lymphoproliferative disorders like MGUS, multiple myeloma & Waldenstrom’s macroglobulinemia

Question 2

In which of the following diseases, platelets are deficient or defective in Gp IIb/IIIa complex ?

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

Question 3

In which of the following diseases, platelets have deficiency or dysfunction of the Gp Ib/IX complex ?

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

Question 3 Explanation:

Inherited platelet function qualitative disorders include autosomal recessive disorders like Glanzmann’s thrombasthenia (absence of platelet GpIIbIIIa receptor) & Bernard Soulier syndrome (absence of platelet GpIb-IX-V receptor). Both present with bleeding symptoms in childhood.

Question 4

Which of the following is not an autosomal recessive trait ?

A	Bernard-Soulier syndrome
B	Glanzmann’s thrombasthenia
C	von Willebrand’s disease Type II
D	von Willebrand’s disease Type III

Question 5

In Heyde’s syndrome, which of the following is a feature apart from aortic stenosis ?

A	Raynaud’s phenomenon
B	Gastrointestinal bleeding
C	Clubbing
D	Pes cavus

Question 5 Explanation:

Heyde’s syndrome refers to aortic stenosis with GI bleeding due to angiodysplasia of GI tract

Question 6

Which of the following platelet aggregation agonists require fibrinogen for binding ?

A	Adenosine diphosphate (ADP)
B	Thrombin
C	Epinephrine
D	All of the above

Question 6 Explanation:

Platelet activation & then aggregation occurs after platelet adhesion. This process is promoted by humoral mediators (epinephrine, thrombin), mediators from activated platelets (ADP, serotonin) & vessel wall extracellular matrix constituents in contact with adherent platelets (collagen, vWF).

Question 7

In platelet granules, which of the following is “adhesive glycoprotein” ?

A	Thrombospondin
B	Fibronectin
C	vWF
D	All of the above

Question 8

von Willebrand factor gene is located on which chromosome ?

A	10
B	11
C	12
D	13

Question 8 Explanation:

von Willebrand factor is a large multimeric glycoprotein that is encoded by a gene spanning 178 kb of genomic DNA on chromosome 12

Question 9

Most common type of vWD is ?

A	Type 1
B	Type 2A
C	Type 2B
D	Type 3

Question 9 Explanation:

vWD is classified into three major types 1, 2 and 3. Type 2 has four subtypes. The most common type of vWD is type 1 disease (80%)

Question 10

Which of the following vWD is inherited as autosomal recessive trait ?

A	Type 1
B	Type 2A
C	Type 2B
D	Type 3

Question 10 Explanation:

Type 3 vWD, which accounts for 1 - 5 % percent of cases, is transmitted as an autosomal recessive trait in homozygous or compound heterozygous persons

Question 11

vWF protein levels are lowest in which of the following blood groups ?

A	A
B	B
C	AB
D	O

Question 11 Explanation:

Patients with “O” blood group have vWF protein levels about one-half those of patients with AB blood type

Question 12

Which of the following is called “severe vWD” ?

A	Type 1
B	Type 2A
C	Type 2B
D	Type 3

Question 12 Explanation:

Type 3 vWD refers to severe vWD - patients with virtually no vWF antigen and FVIII levels <10%.

Question 13

Which type of vWD reflects mutations in vWF that preclude binding of FVIII ?

A	Type 2A
B	Type 2B
C	Type 2M
D	Type 2N

Question 13 Explanation:

Type 2N vWD reflects mutations in vWF that preclude binding of FVIII

Question 14

Which type of vWD is termed “autosomal hemophilia” ?

A	Type 2A
B	Type 2B
C	Type 2M
D	Type 2N

Question 14 Explanation:

Type 2N

Question 15

Acquired vWD is seen most commonly in ?

A	Monoclonal gammopathies of undetermined significance
B	Multiple myeloma
C	Waldenstrom’s macroglobulinemia
D	None of the above

Question 15 Explanation:

Acquired vWD is seen most commonly in MGUS

Question 16

Which of the following best relates to Heyde’s syndrome ?

A	Cirrhosis of liver
B	Angiodysplasia of gastrointestinal tract
C	Pancreatic neoplasis
D	Ectopic spleen

Question 16 Explanation:

Heyde’s syndrome refers to aortic stenosis with gastrointestinal bleeding due to presence of angiodysplasia of gastrointestinal tract in patients with aortic stenosis. Shear stress on blood passing through stenotic aortic valve produces change in vWF, making it susceptible to serum proteases with loss of large multimer forms, leading to acquired type 2 vWD

Question 17

Which of the following is false in von Willebrand Disease ?

A	Prolonged BT
B	Normal platelet count
C	Increased APTT
D	Decreased plasma vWF concentration

Question 17 Explanation:

Laboratory findings in vWD are variable. Most common diagnostic pattern is prolonged BT, normal platelet count, CT, PT, increased APTT, decreased plasma vWF concentration, reduction in its vWF biologic activity (ristocetin cofactor assay) and reduced FVIII activity. Bleeding time is normal in hemophilia A.

Question 18

Platelet count are modestly reduced in which of the following types of vWD ?

A	Type 1
B	Type 2A
C	Type 2B
D	Type 3

Question 18 Explanation:

Platelet counts are within the normal range in vWD except in type 2B vWD where mild thrombocytopenia may be seen and may be misdiagnosed as autoimmune thrombocytopenia. RIPA (Ristocetin induced platelet aggregation) shows a low to absent response in all vWD types except type 2B vWD where a brisk platelet aggregation is seen even at low concentrations

Question 19

What is the nature of “Ristocetin” ?

A	Analgesic
B	Antibiotic
C	Uterine relaxant
D	Digestive enzyme

Question 20

In which of the following vWD, vWF: Ag is normal ?

A	Type 1
B	Type 2A
C	Type 2B
D	Type 3

Question 21

In which of the following vWD, vWF: Ag is normal ?

A	Type 2M
B	Type 2N
C	Type 2B
D	Type 3

Question 21 Explanation:

Normal vWF:Ag levels do not exclude vWD. vWF levels are just below normal in type 1, normal in type 2A and 2M, usually decreased in 2B and 2N and nearly to completely absent in type 3 vWD.

Question 22

Desmopressin acts through which of the following vasopressin receptors ?

A	Type 1
B	Type 2
C	Type 3
D	Type 4

Question 22 Explanation:

Desmopressin is a synthetic derivative of antidiuretic hormone that acts through type 2 vasopressin receptors. It has no activity on type 1 receptors.

Question 23

Dose of Desmopressin is ?

A	0.3 µg / kg body weight IV infusion
B	3.0 µg / kg body weight IV infusion
C	30 µg / kg body weight IV infusion
D	300 µg / kg body weight IV infusion

Question 23 Explanation:

Desmopressin is given in a dose of 0.3 µg / kg body weight IV infusioninfusion for 30 minutes.

Question 24

DDAVP, or desmopressin is most effective for treatment of which type of von Willebrand disease ?

A	Type 1
B	Type 2A
C	Type 2B
D	Type 3

Question 24 Explanation:

Type 1 vWD is best treated with DDAVP (desmopressin), which results in release of vWF and FVIII from endothelial stores. DDAVP is a synthetic hormone given IV injection or by nasal spray.

Question 25

DDAVP, or desmopressin is not indicated in which type of von Willebrand disease ?

A	Type 1
B	Type 2A
C	Type 2B
D	Type 2M

Question 25 Explanation:

Desmopressin or DDAVPis not recommended in type 2B where it is known to aggravate thrombocytopenia.

Question 26

Antifibrinolytic amino acids like Aminocaproic acid or tranexamic acid used in treatment of vWD can cause which of the following complications ?

A	Cholelithiasis
B	Ureteral obstruction
C	Glaucoma
D	Alopecia

Question 26 Explanation:

Antifibrinolytic amino acids are contraindicated in patients with upper UTI or gross hematuria because clots that do not lyse may cause ureteral obstruction.

Question 27

In type 3 vWD, which of the following is the treatment of first choice in patients with alloantibodies ?

A	Desmopressin
B	Factor VIII–von Willebrand factor concentrates
C	Recombinant factor VIII
D	Platelet concentrates

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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. vWD may be acquired in which of the following situations ?
A. Monoclonal gammopathies of undetermined significance
B. Waldenstrom’s macroglobulinemia
C. Multiple myeloma
D. All of the above

2. In which of the following diseases, platelets are deficient or defective in Gp IIb/IIIa complex ?
A. Bernard-Soulier syndrome
B. Glanzmann’s thrombasthenia
C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome

3. In which of the following diseases, platelets have deficiency or dysfunction of the Gp Ib/IX complex ?
A. Bernard-Soulier syndrome
B. Glanzmann’s thrombasthenia

C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome

4. Which of the following is not an autosomal recessive trait ?
A. Bernard-Soulier syndrome
B. Glanzmann’s thrombasthenia
C. von Willebrand’s disease Type II
D. von Willebrand’s disease Type III

5. In Heyde’s syndrome, which of the following is a feature apart from aortic stenosis ?
A. Raynaud’s phenomenon
B. Gastrointestinal bleeding
C. Clubbing
D. Pes cavus

6. Which of the following platelet aggregation agonists require fibrinogen for binding ?
A. Adenosine diphosphate (ADP)
B. Thrombin
C. Epinephrine
D. All of the above

7. In platelet granules, which of the following is “adhesive glycoprotein” ?
A. Thrombospondin
B. Fibronectin
C. vWF
D. All of the above

8. von Willebrand factor gene is located on which chromosome ?
A. 10
B. 11
C. 12
D. 13

9. Most common type of vWD is ?
A. Type 1
B. Type 2A
C. Type 2B

D. Type 3

10. Which of the following vWD is inherited as autosomal recessive trait ?
A. Type 1
B. Type 2A
C. Type 2B
D. Type 3

11. vWF protein levels are lowest in which of the following blood groups ?
A. A
B. B
C. AB
D. O

12. Which of the following is called “severe vWD” ?
A. Type 1
B. Type 2A
C. Type 2B
D. Type 3

13. Which type of vWD reflects mutations in vWF that preclude binding of FVIII ?
A. Type 2A
B. Type 2B
C. Type 2M
D. Type 2N

14. Which type of vWD is termed “autosomal hemophilia” ?
A. Type 2A
B. Type 2B
C. Type 2M
D. Type 2N

15. Acquired vWD is seen most commonly in ?
A. Monoclonal gammopathies of undetermined significance
B. Multiple myeloma

C. Waldenstrom’s macroglobulinemia
D. None of the above

16. Which of the following best relates to Heyde’s syndrome ?
A. Cirrhosis of liver
B. Angiodysplasia of gastrointestinal tract
C. Pancreatic neoplasis
D. Ectopic spleen

17. Which of the following is false in von Willebrand Disease ?
A. Prolonged BT
B. Normal platelet count
C. Increased APTT
D. Decreased plasma vWF concentration

18. Platelet count are modestly reduced in which of the following types of vWD ?
A. Type 1
B. Type 2A
C. Type 2B
D. Type 3

19. What is the nature of “Ristocetin” ?
A. Analgesic
B. Antibiotic
C. Uterine relaxant
D. Digestive enzyme

20. In which of the following vWD, vWF: Ag is normal ?
A. Type 1
B. Type 2A
C. Type 2B
D. Type 3

21. In which of the following vWD, vWF: Ag is normal ?
A. Type 2M
B. Type 2N
C. Type 2B

D. Type 3

22. Desmopressin acts through which of the following vasopressin receptors ?
A. Type 1
B. Type 2
C. Type 3
D. Type 4
.
23. Dose of Desmopressin is ?
A. 0.3 µg / kg body weight IV infusion
B. 3.0 µg / kg body weight IV infusion
C. 30 µg / kg body weight IV infusion
D. 300 µg / kg body weight IV infusion

24. DDAVP, or desmopressin is most effective for treatment of which type of von Willebrand disease ?
A. Type 1
B. Type 2A
C. Type 2B
D. Type 3

25. DDAVP, or desmopressin is not indicated in which type of von Willebrand disease ?
A. Type 1
B. Type 2A
C. Type 2B
D. Type 2M

26. Antifibrinolytic amino acids like Aminocaproic acid or tranexamic acid used in treatment of vWD can cause which of the following complications ?
A. Cholelithiasis
B. Ureteral obstruction
C. Glaucoma
D. Alopecia

27. In type 3 vWD, which of the following is the treatment of first choice in patients with alloantibodies ?
A. Desmopressin

B. Factor VIII–von Willebrand factor concentrates
C. Recombinant factor VIII
D. Platelet concentrates

Disorders of Platelets and Vessel Wall

[MCQs] Disorders of Platelets and Vessel Wall- Part 4

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Disorders of Platelets and Vessel Wall- Part 4

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