[MCQs] Hemolytic Anemias and Anemia Due to Acute Blood Loss - Part 2

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Hemolytic Anemias and Anemia Due to Acute Blood Loss- Part 2

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Question 1

Cold Autoimmune Hemolytic Anemia (AIHA) is caused by ?

A	IgA antibody
B	IgG antibody
C	IgM antibody
D	ANy of the above

Question 1 Explanation:

AIHA is usually classified on the basis of thermal amplitude of the autoantibody. Warm AIHA is caused by IgG antibody, whereas cold AIHA is caused by an IgM antibody that fixes complement to the surface of the RBC

Question 2

Conditions associated with hemolysis and a negative DAT result include all except ?

A	Hemoglobinopathies
B	Systemic lupus erythematosus (SLE)
C	Thrombotic thrombocytopenic purpura (TTP)
D	Disseminated intravascular coagulation (DIC)

Question 2 Explanation:

Antierythrocyte membrane autoantibodies in SLE have positive direct Coombs’ test (60% prevalence). Some may develop overt hemolysis. Conditions associated with hemolysis and a negative DAT result are Microangiopathic hemolytic anemias (TTP, DIC), Hypersplenism, Liver disease, Hemoglobinopathies (sickle cell disease, thalassemia), Erythrocyte membranopathies (spherocytosis), Erythrocyte enzymopathies (G-6-PD deficiency, pyruvate kinase deficiency), Infectious diseases (Clostridium difficile infection), Erythrocyte trauma (mechanical heart valves).

Question 3

RBC’s of which blood group are used in indirect antiglobulin test (IAT) ?

A	A
B	B
C	AB
D	O

Question 4

Which of the following should be considered in differential diagnosis of chronic Coombs-negative HA ?

A	Drug-induced hemolytic anemias
B	Enzymopathies
C	Paroxysmal Cold Hemoglobinuria (PCH)
D	Acute hemolytic transfusion reaction

Question 4 Explanation:

Enzymopathies (G-6-PD deficiency, pyruvate kinase deficiency) should be considered in the differential diagnosis of any chronic Coombs-negative HA.

Question 5

Most common form of acquired hemolytic anemia in areas where malaria is not endemic is ?

A	Paroxysmal Nocturnal Hemoglobinuria (PNH)
B	Paroxysmal Cold Hemoglobinuria (PCH)
C	Autoimmune Hemolytic Anemia (AIHA)
D	Hemolytic Uremic Syndrome (HUS)

Question 5 Explanation:

Autoimmune Hemolytic Anemia (AIHA) is the most common form of acquired hemolytic anemia in areas where malaria is not endemic.

Question 6

Paroxysmal cold hemoglobinuria (PCH) was more frequent when which of the following disease was prevalent ?

A	Tuberculosis
B	Tertiary syphilis
C	Gonorrhoea
D	Protein calorie malnutrition

Question 7

Antibody in Paroxysmal cold hemoglobinuria (PCH) is ?

A	Anti-Jo-1 antibody
B	Anti-Smith antibody
C	Ki-67 monoclonal antibody
D	Donath-Landsteiner antibody

Question 7 Explanation:

Antibody in PCH is Donath-Landsteiner antibody which has anti-P specificity and binds to RBC’s only at a low temperature (~4°C), and in the presence of complement when temperature becomes 37°C, lysis of RBC’s, intravascular hemolysis & hemoglobinuria occurs.

Question 8

Cold Agglutinin Disease (CAD) is related to which of the following ?

A	Familial cold autoinflammatory syndrome (FCAS)
B	Cold urticaria
C	Waldenström macroglobulinemia (WM)
D	Systemic Mastocytosis

Question 8 Explanation:

CAD is a form of chronic AIHA & affects elderly. Autoantibody (IgM with anti-I specificity) reacts with RBC’s strongly at lower temperatures (cold exposure), not at all at 37°C. Antibody is produced by expanded clone of B lymphocytes and may show as a spike in plasma protein electrophoresis resembling monoclonal gammopathy and may be related to Waldenström macroglobulinemia (WM).

Question 9

Which of the following is the most important protective RBC membrane protein ?

A	CD29
B	CD39
C	CD49
D	CD59

Question 9 Explanation:

CD59 (membrane inhibitor of reactive lysis) is a protective RBC membrane protein.

Question 10

Which of the following is diagnostic of PNH ?

A	CD59–, CD55–
B	CD59+, CD55–
C	CD59–, CD55+
D	CD59+, CD55+

Question 10 Explanation:

Discrete population of cells that is CD59–, CD55– is diagnostic of PNH.

Question 11

Which of the following is the action of CD59 ?

A	Inhibits insertion of C9 into cell membrane
B	Binds C3b
C	Inhibiting C3 convertases
D	All of the above

Question 11 Explanation:

Erythrocytes defend themselves from membrane attack complexes with two membrane-bound proteins, CD55 (decay-accelerating factor), which binds C3b, and CD59, which inhibits the insertion of C9 into the membrane

Question 12

Which of the following is false about Paroxysmal Nocturnal Hemoglobinuria (PNH) ?

A	Intracorpuscular defect acquired at stem cell level
B	Hemolytic anemia
C	Arterial thrombosis
D	Bone marrow failure

Question 12 Explanation:

PNH has three attributes - complement-dependent acquired chronic intravascular hemolytic anemia, venous thrombosis & bone marrow failure. Hemolysis often occurs during sleep, when decline in blood pH triggers activation of complement components. Urine is black when patient awakens. Hemoglobin-drenched renal tubules lock iron of Hb into hemosiderin and continuous sloughing of these cells into urine culminates in iron deficiency.

Question 13

Which of the following is false about Paroxysmal Nocturnal Hemoglobinuria (PNH) ?

A	Hemolysis due to activation of complement
B	Hemosiderinuria is usually absent
C	Granulocytopenia & thrombocytopenia
D	May present as Budd-Chiari syndrome

Question 13 Explanation:

In PNH, RBC’s have an increased susceptibility to complement (C) due to the deficiency on their surface of CD59 & CD55 proteins that normally protect RBC’s from activated C. When thrombosis affects hepatic veins, Budd-Chiari syndrome occurs. The hemoglobin-drenched renal tubules lock iron of hemoglobin into hemosiderin, and the continuous sloughing of these cells into the urine culminates in iron deficiency.

Question 14

In Paroxysmal Nocturnal Hemoglobinuria, name of the defective gene is ?

A	HNF4
B	PIG-A
C	BRCA1
D	CFTR

Question 14 Explanation:

PNH results from clonal expansion of hematopoietic stem cells that have somatic mutations in the X-linked gene called PIG-A (phosphatidylinositol glycan class A). PIG-A mutations cause an early block in the synthesis of glycosylphosphatidyl-inositol (GPI) anchors, which tether many proteins to the cell surface. Consequently, the blood cells in PNH have a partial deficiency (type II) or a complete deficiency (type III) of GPI-linked proteins. Intravascular hemolysis is the consequence of absence of GPI-linked complement regulatory protein CD59. CD59 blocks the formation of terminal complement complex (membrane-attack complex) on the cell surface, thereby preventing erythrocyte lysis and in vitro platelet activation.

Question 15

Which of the following tests is useful in diagnosing Paroxysmal Nocturnal Hemoglobinuria ?

A	Acidified serum lysis test (Ham’s test)
B	Sucrose lysis test
C	Analysis of GPI-linked proteins (CD59, DAF)
D	All of the above

Question 15 Explanation:

Thomas Hale Ham (1938) developed Ham’s test at Thorndike Laboratory of Boston City Hospital and reported that RBC’s in PNH are highly susceptible to lysis in an acidic environment. CD55 is called decay-accelerating factor (DAF)

Question 16

Pathway of activation of the complement system is called ?

A	Classical pathway
B	Mannose-binding lectin pathway
C	Alternative pathway
D	All of the above

Question 16 Explanation:

There are three pathways of activation of the complement system - the classical, mannosebinding lectin and alternative pathways. Terminal pathway is common to these three pathways leads to membrane attack complex that lyses cells.

Question 17

Pathways of activation of the complement system converge at the point of cleavage of ?

A	C3
B	C4
C	C5
D	C6

Question 17 Explanation:

3 activation pathways of complement converge to generate C3 convertase that cleaves C3

Question 18

Eculizumab binds specifically to ?

A	C1
B	C3
C	C4
D	C5

Question 18 Explanation:

Eculizumab is a recombinant humanized monoclonal antibody that binds specifically to terminal complement protein C5, inhibiting its cleavage into C5a and C5b, thereby preventing release of inflammatory mediator C5a & formation of the cytolyticpore C5b–C9. Blockade of complement cascade at C5 preserves early components of complement that are essential for opsonization of microorganisms & clearance of immune complexes

Question 19

RBC membrane is unstable at temperatures above ?

A	38 ºC
B	42 ºC
C	46 ºC
D	49 ºC

Question 19 Explanation:

RBC membrane is unstable at temperatures > 49°C due to denaturation of cytoskeletal protein spectrin

Question 20

In hemolysis, level of unconjugated bilirubin never exceeds ?

A	1 to 2 mg / dL
B	2 to 3 mg / dL
C	3 to 4 mg / dL
D	4 to 5 mg / dL

Question 20 Explanation:

In patients with hemolysis, the level of unconjugated bilirubin never exceeds 4 to 5 mg/dL unless liver function is impaired

Question 21

Which fraction of lactate dehydrogenase (LDH) is elevated by accelerated RBC destruction ?

A	1
B	2
C	3
D	4

Question 21 Explanation:

LDH, particularly LDH-2, is elevated by accelerated RBC destruction. Serum AST (SGOT) may be elevated but ALT (SGPT) is not.

Question 22

Which of the following is false about ‘Haptoglobin’ ?

A	 globulin
B	Binds to heme in hemoglobin
C	Decreased in hepatocellular disease
D	Increased in inflammatory states

Question 22 Explanation:

Haptoglobin binds specifically to the globin in hemoglobin

Question 23

Which of the following is false about ‘Haptoglobin’ ?

A	Serum concentration is ~1.0 g/L
B	Low or absent is significant hemolysis
C	Hemoglobin-haptoglobin complex is cleared rapidly by mononuclear phagocyte system
D	None of the above

Question 24

Urine is positive with benzidine reaction in ?

A	Hemoglobinuria
B	Hematuria
C	Myoglobinuria
D	All of the above

Question 24 Explanation:

Urine is positive with benzidine reaction in hemoglobinuria, hematuria and myoglobinuria

Question 25

Which of the following stains is used to detect hemosiderin in urinary sediment ?

A	Prussian blue
B	Giemsa’s stain
C	Methylene blue
D	Crystal violet

Question 25 Explanation:

Prussian blue is used to detect presence of hemosiderin in urine. Positive result indicates that a significant amount of circulating free hemoglobin has been filtered by the kidneys.

Question 26

Drug that cause immunohemolytic anemia is ?

A	-methyldopa
B	Penicillin
C	Quinidine
D	All of the above

Question 26 Explanation:

Drugs causing warm antibody immunohemolytic anemia are -methyldopa, penicillin & quinidine

Question 27

Which of the following can directly parasitize RBC’s & cause severe hemolysis ?

A	Bartonellosis
B	Malaria
C	Babesiosis
D	All of the above

Question 27 Explanation:

Bartonellosis, malaria & babesiosis directly parasitize RBC & cause severe hemolysis

Question 28

Spur cell anemia is due to ?

A	Clostridium welchii infection
B	HELLP syndrome
C	Severe hepatocellular disease
D	Hemolytic-uremic syndrome

Question 28 Explanation:

Spur cells are irregularly spiculated acanthocytes. Spur cell anemia is a hemolytic anemia with bizarre-shaped RBC occuring in severe hepatocellular disease (Laennec’s cirrhosis)

Question 29

Surface membrane of a spur cell (acanthocytes) contain excess of ?

A	Phospholipid
B	Cholesterol
C	Triglyceride
D	Glycoprotein

Question 29 Explanation:

Surface membrane of a spur cell contains 50 to 70% excess cholesterol, but its total phospholipid content is normal. This decreases membrane fluidity & cell deformability forbiding their passage through filtering system of spleen.

Question 30

Burr cells’ are found in ?

A	Severe hepatocellular disease
B	Uremia
C	DIC
D	HELLP syndrome

Question 30 Explanation:

Burr cells or echinocytes are seen in uremia. In such RBC’s numerous, regularly spaced, small spiny projections are seen. Echinocytes are a frequent artifact in PBF.

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Hemolytic Anemias and Anemia Due to Acute Blood Loss – Part 1 | Hemolytic Anemias and Anemia Due to Acute Blood Loss – Part 2

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1. Cold Autoimmune Hemolytic Anemia (AIHA) is caused by ?
A. IgA antibody
B. IgG antibody
C. IgM antibody
D. ANy of the above

2. Conditions associated with hemolysis and a negative DAT result include all except ?-1
A. Hemoglobinopathies
B. Systemic lupus erythematosus (SLE)
C. Thrombotic thrombocytopenic purpura (TTP)
D. Disseminated intravascular coagulation (DIC)

3. RBC’s of which blood group are used in indirect antiglobulin test (IAT) ?
A. A
B. B
C. AB
D. O
4. Which of the following should be considered in differential diagnosis of chronic Coombs-negative HA ?
A. Drug-induced hemolytic anemias
B. Enzymopathies
C. Paroxysmal Cold Hemoglobinuria (PCH)
D. Acute hemolytic transfusion reaction

5. Most common form of acquired hemolytic anemia in areas where malaria is not endemic is ?
A. Paroxysmal Nocturnal Hemoglobinuria (PNH)
B. Paroxysmal Cold Hemoglobinuria (PCH)
C. Autoimmune Hemolytic Anemia (AIHA)
D. Hemolytic Uremic Syndrome (HUS)

6. Paroxysmal cold hemoglobinuria (PCH) was more frequent when which of the following disease was prevalent ?
A. Tuberculosis
B. Tertiary syphilis
C. Gonorrhoea
D. Protein calorie malnutrition

7. Antibody in Paroxysmal cold hemoglobinuria (PCH) is ?
A. Anti-Jo-1 antibody
B. Anti-Smith antibody
C. Ki-67 monoclonal antibody
D. Donath-Landsteiner antibody

8. Cold Agglutinin Disease (CAD) is related to which of the following ?
A. Familial cold autoinflammatory syndrome (FCAS)
B. Cold urticaria
C. Waldenström macroglobulinemia (WM)
D. Systemic Mastocytosis

9. Which of the following is the most important protective RBC membrane protein ?
A. CD29
B. CD39
C. CD49
D. CD59

10. Which of the following is diagnostic of PNH ?
A. CD59–, CD55–
B. CD59+, CD55–
C. CD59–, CD55+
D. CD59+, CD55+

11. Which of the following is the action of CD59 ?
A. Inhibits insertion of C9 into cell membrane
B. Binds C3b
C. Inhibiting C3 convertases
D. All of the above

12. Which of the following is false about Paroxysmal Nocturnal Hemoglobinuria (PNH) ?

A. Intracorpuscular defect acquired at stem cell level
B. Hemolytic anemia
C. Arterial thrombosis
D. Bone marrow failure

13. Which of the following is false about Paroxysmal Nocturnal Hemoglobinuria (PNH) ?
A. Hemolysis due to activation of complement
B. Hemosiderinuria is usually absent
C. Granulocytopenia & thrombocytopenia
D. May present as Budd-Chiari syndrome

14. In Paroxysmal Nocturnal Hemoglobinuria, name of the defective gene is ?
A. HNF4
B. PIG-A
C. BRCA1
D. CFTR

15. Which of the following tests is useful in diagnosing Paroxysmal Nocturnal Hemoglobinuria ?
A. Acidified serum lysis test (Ham’s test)
B. Sucrose lysis test
C. Analysis of GPI-linked proteins (CD59, DAF)
D. All of the above

16. Pathway of activation of the complement system is called ?
A. Classical pathway
B. Mannose-binding lectin pathway
C. Alternative pathway
D. All of the above

17. Pathways of activation of the complement system converge at the point of cleavage of ?
A. C3
B. C4
C. C5
D. C6

18. Eculizumab binds specifically to ?
A. C1
B. C3
C. C4
D. C5

19. RBC membrane is unstable at temperatures above ?
A. 38 ºC
B. 42 ºC
C. 46 ºC
D. 49 ºC

20. In hemolysis, level of unconjugated bilirubin never exceeds ?
A. 1 to 2 mg / dL
B. 2 to 3 mg / dL
C. 3 to 4 mg / dL
D. 4 to 5 mg / dL

21. Which fraction of lactate dehydrogenase (LDH) is elevated by accelerated RBC destruction ?
A. 1
B. 2
C. 3
D. 4

22. Which of the following is false about ‘Haptoglobin’ ?
A.  globulin
B. Binds to heme in hemoglobin
C. Decreased in hepatocellular disease
D. Increased in inflammatory states

23. Which of the following is false about ‘Haptoglobin’ ?
A. Serum concentration is ~1.0 g/L
B. Low or absent is significant hemolysis
C. Hemoglobin-haptoglobin complex is cleared rapidly by
mononuclear phagocyte system
D. None of the above

24. Urine is positive with benzidine reaction in ?
A. Hemoglobinuria
B. Hematuria
C. Myoglobinuria
D. All of the above

25. Which of the following stains is used to detect hemosiderin in urinary sediment ?
A. Prussian blue
B. Giemsa’s stain
C. Methylene blue
D. Crystal violet

26. Drug that cause immunohemolytic anemia is ?
A. -methyldopa
B. Penicillin
C. Quinidine
D. All of the above

27. Which of the following can directly parasitize RBC’s & cause severe hemolysis ?
A. Bartonellosis
B. Malaria
C. Babesiosis
D. All of the above

28. Spur cell anemia is due to ?
A. Clostridium welchii infection
B. HELLP syndrome
C. Severe hepatocellular disease
D. Hemolytic-uremic syndrome

29. Surface membrane of a spur cell (acanthocytes) contain excess of ?
A. Phospholipid

B. Cholesterol
C. Triglyceride
D. Glycoprotein

30. ‘Burr cells’ are found in ?
A. Severe hepatocellular disease
B. Uremia
C. DIC
D. HELLP syndrome

[MCQs] Hemolytic Anemias and Anemia Due to Acute Blood Loss – Part 2

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Hemolytic Anemias and Anemia Due to Acute Blood Loss- Part 2

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