[MCQs] Disorders of Platelets and Vessel Wall- Part 2

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Disorders of Platelets and Vessel Wall- Part 2

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Question 1

All IVIgG immunoglobulin preparations are derived from ?

A	Human plasma
B	Horse plasma
C	Bovine plasma
D	Pig plasma

Question 1 Explanation:

All IVIgG immunoglobulin preparations are derived from human plasma.

Question 2

In ITP, total dose of IVIgG is ?

A	1 gram / kg
B	2 gram / kg
C	. 3 gram / kg
D	4 gram / kg

Question 2 Explanation:

In ITP, total dose of IVIgG is 2 gram / kg

Question 3

Which of the following drugs is useful in refractory ITP ?

A	Rituximab
B	Romiplostim
C	Eltrombopag
D	All of the above

Question 3 Explanation:

Rituximab, an anti-CD20 (B cell) antibody, has shown efficacy in the treatment of refractory ITP. Two thrombopoietin receptor agonists - one administered subcutaneously (romiplostim) and another orally (eltrombopag), have shown response in many patients with refractory ITP

Question 4

Large platelets is a feature of which of the following ?

A	May-Hegglin anomaly
B	Sebastian syndrome
C	Epstein’s syndrome
D	All of the above

Question 4 Explanation:

May-Hegglin anomaly, and Sebastian, Epstein’s, and Fechtner syndromes have thrombocytopenia with large platelets & are inherited as autosomal dominant & are associated with mutations in nonmuscle myosin heavy chain MYH9 gene.

Question 5

Autosomal recessive inherited thrombocytopenic disorders include ?

A	Congenital amegakaryocytic thrombocytopenia
B	Thrombocytopenia with absent radii
C	Bernard Soulier syndrome
D	All of the above

Question 5 Explanation:

Autosomal recessive inherited thrombocytopenic disorders include congenital amegakaryocytic thrombocytopenia, thrombocytopenia with absent radii and Bernard Soulier syndrome

Question 6

Which of the following is inherited as X-linked pattern in inherited thrombocytopenia ?

A	May-Hegglin anomaly
B	Wiskott-Aldrich syndrome
C	Fechtner syndrome
D	Bernard Soulier syndrome

Question 6 Explanation:

X-linked inherited thrombocytopenic disorders include Wiskott-Aldrich synd

Question 7

Which of the following is false regarding thrombotic thrombocytopenic microangiopathies ?

A	Thrombocytopenia
B	Laboratory evidence of hemolysis
C	Abnormal PT and aPTT
D	Microvascular thrombosis

Question 7 Explanation:

Thrombotic thrombocytopenic microangiopathies are characterized by thrombocytopenia, microangiopathic hemolytic anemia, laboratory evidence of hemolysis & microvascular thrombosis. It includes thrombotic thrombocytopenic purpura (TTP) & hemolytic uremic syndrome (HUS). PT & aPTT are characteristically normal in TTP or HUS and is used to differentiate TTP from DIC. Despite thrombocytopenia, severe bleeds are uncommon. Neurologic changes are common in TTP, renal failure in HUS & neurologic or renal changes are absent in HELLP.

Question 8

Thrombotic thrombocytopenic purpura (TTP) was first described by ?

A	Balint
B	Eli Moschcowitz
C	Verner-Morrison
D	Wiskott-Aldrich

Question 8 Explanation:

TTP was initially described by Dr Eli Moschcowitz at the Mount Sinai Hospital in New York City in 1924. Thrombotic thrombocytopenic purpura (TTP) is also called Moschcowitz disease.

Question 9

Which of the following is not a feature of TTP ?

A	Microangiopathic hemolytic anemia
B	Renal failure
C	Hepatic failure
D	Neurologic dysfunction

Question 10

Upshaw-Schulman syndrome is related to ?

A	Multiple transfusions
B	Thrombotic thrombocytopenic purpura
C	Uremia
D	CHF

Question 10 Explanation:

Upshaw Schulman syndrome is due to a hereditary deficiency of a metalloprotease that cleaves vWF which is normally secreted as ultra-large multimers. It is cleaved by ADAMTS13. Persistence of ultra-large vWF molecules produces pathogenic platelet adhesion & aggregation

Question 11

Upshaw-Schulman syndrome is best related to ?

A	Idiopathic TTP
B	Inherited TTP
C	Medication-related TTP
D	TTP in pregnant women

Question 11 Explanation:

Hereditary form of TTP is called Upshaw-Schulman syndrome. It is due to inherited deficiency of or antibodies to the plasma metalloprotease ADAMTS13. 5-10% of all TTP cases are due to Upshaw-Schulman syndrome.

Question 12

Idiopathic TTP is more common in ?

A	Women
B	HIV infection
C	Pregnant women
D	All of the above

Question 12 Explanation:

Idiopathic TTP is more common in women, HIV infection & in pregnant women

Question 13

Which of the following antiplatelet agent has been implicated in the causation of TTP ?

A	Aspirin
B	Ticlopidine
C	Carvadilol
D	All of the above

Question 13 Explanation:

Medication-related TTP may be secondary to antibody formation (ticlopidine) or direct endothelial toxicity (cyclosporine, mitomycin C, tacrolimus, quinine).

Question 14

In TTP, hyaline thrombi without inflammatory changes in vessel wall may be found in ?

A	Arterioles
B	Capillaries
C	Venules
D	All of the above

Question 14 Explanation:

Presence of hyaline thrombi in arterioles, capillaries & venules without any inflammatory changes in the vessel wall is diagnostic of TTP

Question 15

Severity of TTP is estimated by the degree of ?

A	Anemia
B	Thrombocytopenia
C	Serum LDH level
D	All of the above

Question 16

Which of the following is not a feature of TTP ?

A	Severe Coombs-positive hemolytic anemia
B	Near normal prothrombin time
C	Near normal fibrinogen level
D	Near normal FDP level

Question 16 Explanation:

Presence of severe Coombs-negative hemolytic anemia with schistocytes or fragmented RBC’s in PBF, with thrombocytopenia & minimal activation of coagulation system confirm diagnosis of TTP

Question 17

Which of the following supports the diagnosis of TTP ?

A	Increased lactate dehydrogenase
B	Increased indirect bilirubin
C	Decreased haptoglobin
D	All of the above

Question 17 Explanation:

Findings that support the diagnosis of TTP include increased LDH, increased indirect bilirubin, decreased haptoglobin, increased reticulocyte count, negative direct antiglobulin test, schistocytes in PBF and polychromasia.

Question 18

Mainstay of treatment of idiopathic TTP is ?

A	Rituximab
B	IV Ig
C	Plasma exchange
D	Splenectomy

Question 18 Explanation:

Plasma exchange remains the mainstay of treatment of ITP idiopathic TTP.

Question 19

Which of the following is false about TTP ?

A	TTP is more common in HIV patients and pregnant women
B	Coagulation studies are essentially normal in TTP
C	Severe bleeds are usually absent
D	None of the above

Question 20

Which of the following is false about Weibel-Palade bodies ?

A	Are organelles in the vascular endothelium
B	Major constituents of Weibel-Palade bodies are von Willebrand factor (vWF) and P-selectin
C	George Emil Palade won the Nobel Prize in Physiology or Medicine in 1974
D	None of the above

Question 21

Which of the following is false about Weibel-Palade bodies (WPBs) ?

A	Weibel-Palade bodies play a dual role in blood coagulation hemostasis and inflammation
B	Weibel-Palade bodies are the main source of vWF
C	Weibel-Palade bodies are secretory organelles used for post-synthesis storage in endothelial cells.
D	None of the above

Question 22

Which of the following is false about Weibel-Palade bodies (WPBs) ?

A	Weibel-Palade bodies play a dual role in blood coagulation hemostasis and inflammation
B	Weibel-Palade bodies are the main source of vWF
C	Weibel-Palade bodies are secretory organelles used for post-synthesis storage in endothelial cells.
D	None of the above

Question 23

TTP is due to deficiency in the activity of ?

A	ADAMTS 11
B	ADAMTS 12
C	ADAMTS 13
D	ADAMTS 14

Question 23 Explanation:

ADAMTS 13 is a member of ADAMTS (a disintegrin with thrombospondin type 1 motifs) zinc metalloproteinase family that cleaves vWF complexes & prevents vWF-platelet interaction. Severe deficiency of ADAMTS causes TTP.

Question 24

Which of the following is related to TTP ?

A	ADAMTS-10
B	ADAMTS-11
C	ADAMTS-12
D	ADAMTS-13

Question 24 Explanation:

Plasma metalloprotease ADAMTS-13 (vWF-cleaving protease) is responsible for proteolytic cleavage of large hemostatically hyperactive vWf multimers that are synthesized and secreted by endothelial cells in plasma into smaller less adhesive multimers. ADAMTS-13 prevents inappropriate microvascular platelet aggregation. Deficiency of ADAMTS-13 results in TTP. The relationship of reduced ADAMTS13 to the pathogenesis of TTP is known as the Furlan-Tsai hypothesis

Question 25

Which of the following is false about ADAMTS-13 ?

A	ADAMTS-13 prevents inappropriate microvascular platelet aggregation
B	Deficiency of ADAMTS-13 is a finding for TTP
C	vWf multimers is the physiologic substrate for ADAMTS-13
D	None of the above

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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. All IVIgG immunoglobulin preparations are derived from ?
A. Human plasma
B. Horse plasma
C. Bovine plasma
D. Pig plasma

2. In ITP, total dose of IVIgG is ?
A. 1 gram / kg
B. 2 gram / kg
C. 3 gram / kg
D. 4 gram / kg

3. Which of the following drugs is useful in refractory ITP ?
A. Rituximab
B. Romiplostim
C. Eltrombopag
D. All of the above

4. Large platelets is a feature of which of the following ?
A. May-Hegglin anomaly
B. Sebastian syndrome
C. Epstein’s syndrome
D. All of the above

5. Autosomal recessive inherited thrombocytopenic disorders include ?
A. Congenital amegakaryocytic thrombocytopenia
B. Thrombocytopenia with absent radii
C. Bernard Soulier syndrome
D. All of the above

6. Which of the following is inherited as X-linked pattern in inherited thrombocytopenia ?
A. May-Hegglin anomaly
B. Wiskott-Aldrich syndrome
C. Fechtner syndrome
D. Bernard Soulier syndrome

7. Which of the following is false regarding thrombotic thrombocytopenic microangiopathies ?
A. Thrombocytopenia
B. Laboratory evidence of hemolysis
C. Abnormal PT and aPTT
D. Microvascular thrombosis

8. Thrombotic thrombocytopenic purpura (TTP) was first described by ?
A. Balint
B. Eli Moschcowitz
C. Verner-Morrison
D. Wiskott-Aldrich

9. Which of the following is not a feature of TTP ?
A. Microangiopathic hemolytic anemia
B. Renal failure
C. Hepatic failure
D. Neurologic dysfunction

10. Upshaw-Schulman syndrome is related to ?
A. Multiple transfusions
B. Thrombotic thrombocytopenic purpura

C. Uremia
D. CHF

11. Upshaw-Schulman syndrome is best related to ?
A. Idiopathic TTP
B. Inherited TTP
C. Medication-related TTP
D. TTP in pregnant women

12. Idiopathic TTP is more common in ?
A. Women
B. HIV infection
C. Pregnant women
D. All of the above

13. Which of the following antiplatelet agent has been implicated in the causation of TTP ?
A. Aspirin
B. Ticlopidine
C. Carvadilol
D. All of the above

14. In TTP, hyaline thrombi without inflammatory changes in vessel wall may be found in ?
A. Arterioles
B. Capillaries
C. Venules
D. All of the above

15. Severity of TTP is estimated by the degree of ?
A. Anemia
B. Thrombocytopenia
C. Serum LDH level
D. All of the above

16. Which of the following is not a feature of TTP ?
A. Severe Coombs-positive hemolytic anemia
B. Near normal prothrombin time
C. Near normal fibrinogen level

D. Near normal FDP level

17. Which of the following supports the diagnosis of TTP ?
A. Increased lactate dehydrogenase
B. Increased indirect bilirubin
C. Decreased haptoglobin
D. All of the above

18. Mainstay of treatment of idiopathic TTP is ?
A. Rituximab
B. IV Ig
C. Plasma exchange
D. Splenectomy

19. Which of the following is false about TTP ?
A. TTP is more common in HIV patients and pregnant women
B. Coagulation studies are essentially normal in TTP
C. Severe bleeds are usually absent
D. None of the above

20. Which of the following is false about Weibel-Palade bodies ?
A. Are organelles in the vascular endothelium
B. Major constituents of Weibel-Palade bodies are von Willebrand factor (vWF) and P-selectin
C. George Emil Palade won the Nobel Prize in Physiology or Medicine in 1974
D. None of the above

21. Which of the following is false about Weibel-Palade bodies (WPBs) ?
A. Weibel-Palade bodies play a dual role in blood coagulation hemostasis and inflammation
B. Weibel-Palade bodies are the main source of vWF
C. Weibel-Palade bodies are secretory organelles used for post-synthesis storage in endothelial cells.
D. None of the above

22. TTP is due to deficiency in the activity of ?
A. ADAMTS 11
B. ADAMTS 12
C. ADAMTS 13
D. ADAMTS 14

23. Which of the following is related to TTP ?
A. ADAMTS-10
B. ADAMTS-11
C. ADAMTS-12
D. ADAMTS-13

24. Which of the following is false about ADAMTS-13 ?
A. ADAMTS-13 prevents inappropriate microvascular platelet aggregation
B. Deficiency of ADAMTS-13 is a finding for TTP
C. vWf multimers is the physiologic substrate for ADAMTS-13
D. None of the above

Disorders of Platelets and Vessel Wall

[MCQs] Disorders of Platelets and Vessel Wall- Part 2

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Disorders of Platelets and Vessel Wall- Part 2

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