[MCQs] Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes - Part 1

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Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes- Part 1

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Question 1

Which of the following is not a hypoproliferative anemia ?

A	Aplastic anemia
B	Myelodysplasia (MDS)
C	Pure red cell aplasia (PRCA)
D	Congenital dyserythropoietic anemia

Question 1 Explanation:

Hypoproliferative anemia is a prominent feature of bone marrow failure states like aplastic anemia, myelodysplasia (MDS), pure red cell aplasia (PRCA) and myelophthisis

Question 2

Most cases of aplastic anemia are ?

A	Idiopathic
B	Due to drug exposure
C	Due to immune diseases
D	Due to inherited disorders

Question 2 Explanation:

Most cases of aplastic anemia are idiopathic

Question 3

In aplastic anemia, age affected is ?

A	Children
B	Teens
C	Young adults
D	Teens & twenties and older adults

Question 3 Explanation:

In aplastic anemia, men & women are affected equally. The age distribution is biphasic, with the major peak in the teens and twenties and a second rise in older adults.

Question 4

Which of the following is not a late effect of irradiation ?

A	Aplastic anemia
B	MDS
C	Leukemia
D	All of the above

Question 4 Explanation:

MDS & leukemia, but probably not aplastic anemia, are late effects of radiation

Question 5

Which of the following has consistent association with aplastic anemia ?

A	Benzene
B	Chloramphenicol
C	Carbamazapine
D	All of the above

Question 6

Which of the following has consistent association with aplastic anemia ?

A	Gold
B	Phenylbutazone
C	Cimetidine
D	All of the above

Question 6 Explanation:

Benzene, hydantoins, carbamazapine, quinacrine, gold, cimetidine, chloramphenicol, phenylbutazone show most consistent association with AA.

Question 7

Which of the following hepatitis virus infections most often precede posthepatitis marrow failure ?

A	A
B	B
C	C
D	None of the above

Question 7 Explanation:

Posthepatitis aplastic anemia is typically seronegative (non-A, non-B, non-C, non-G) & probably due to as yet undiscovered infectious agent.

Question 8

Cause of transient aplastic crisis in hemolytic anemias is ?

A	Hepatitis
B	Infectious mononucleosis
C	Parvovirus B19
D	All of the above

Question 8 Explanation:

Parvovirus B19 is the cause of transient aplastic crisis in hemolytic anemias

Question 9

Aplastic anemia is strongly associated with which of the following collagen vascular syndrome ?

A	Polyarteritis nodosa
B	Schnitzler’s syndrome
C	Wegener’s granulomatosis
D	Eosinophilic fasciitis

Question 9 Explanation:

Aplastic anemia is strongly associated with eosinophilic fasciitis which is characterized by painful induration of subcutaneous tissues.

Question 10

Aplastic anemia is strongly associated with which of the following ?

A	Transfusion-associated graft-versus-host disease (GVHD)
B	Eosinophilic fasciitis
C	Systemic lupus erythematosus (SLE)
D	All of the above

Question 11

Aplastic anemia is related to ?

A	Fanconi’s anemia
B	Paroxysmal nocturnal hemoglobinuria
C	Dyskeratosis congenita
D	All of the above

Question 11 Explanation:

Fanconi’s anemia presents as progressive pancytopenia. Bone marrow from PNH patients show evidence of defective hematopoiesis and may later develop frank marrow aplasia & pancytopenia. In Dyskeratosis congenita, aplastic anemia develops during childhood.

Question 12

Which of the following about Fanconi’s anemia is false ?

A	Autosomal dominant disorder
B	Short stature, café au lait spots
C	Type A is due to mutation in FANCA
D	Increased risk of malignancy

Question 12 Explanation:

Fanconi’s anemia is an autosomal recessive disorde

Question 13

Dyskeratosis congenita is characterized by all except ?

A	Abnormal skin pigmentation
B	Pancreatic insufficiency
C	Nail dystrophy
D	Mucosal leucoplakia

Question 13 Explanation:

Dyskeratosis congenita is an inherited bone-marrow-failure syndrome in childhood & presents with the triad of reticular hyperpigmentation, nail dystrophy and mucous membrane leukoplasia

Question 14

Dyskeratosis is due to mutations in which of the following genes ?

A	DKC1
B	TERC
C	TERT
D	Any of the above

Question 14 Explanation:

Dyskeratosis congenita is due to mutations in genes of the telomere repair complex that maintain telomere length in replicating cells: The X-linked variety is due to mutations in DKC1 (dyskerin) gene. the autosomal dominant type is due to mutation in TERC, which encodes an RNA template, and TERT, which encodes the catalytic reverse transcriptase, telomerase. Mutations in TNF2, a component of the shelterin, proteins that bind the telomere DNA, also occur in dyskeratosis.

Question 15

Shwachman-Diamond syndrome features include all except ?

A	Pancreatic insufficiency
B	Malabsorption
C	Eosinophilia
D	Risk of aplastic anemia

Question 15 Explanation:

Schwachman-Diamond syndrome is an autosomal recessive disorder characterised by pancreatic exocrine dysfunction, malabsorption, metaphyseal dysostosis, and bone marrow failure due to compound heterozygous mutations in SBDS.

Question 16

Which cell in the bone marrow is helpful in differentiating aplastic anaemia from hypoplastic myelodysplastic syndromes ?

A	CD34 positive stem cells
B	CD38 positive stem cells
C	CD42 positive stem cells
D	CD46 positive stem cells

Question 16 Explanation:

Bone marrow failure results from severe damage to hematopoietic cell compartment. In AA, cells bearing CD34 antigen, a marker of early hematopoietic cells, are greatly diminished, committed & primitive progenitor cells are virtually absent

Question 17

Which of the following is the most common early symptom in aplastic anemia ?

A	Bleeding
B	Infection
C	Weight loss
D	Jaundice

Question 17 Explanation:

Aplastic anemia can have abrupt or insidious onset. Most common early symptom is bleeding. Systemic complaints and weight loss should point to other etiologies of pancytopenia

Question 18

Which of the following is unusual in aplastic anemia ?

A	Infection on presentation
B	Lymphadenopathy
C	Splenomegaly
D	All of the above

Question 18 Explanation:

Infection on presentation, lymphadenopathy & splenomegaly are atypical of aplastic anemia.

Question 19

Which of the following is not a feature of blood smear in aplastic anemia ?

A	Decreased mean corpuscular volume (MCV)
B	Few or absent reticulocytes
C	Normal lymphocyte number
D	Reduced platelets and granulocytes

Question 19 Explanation:

In AA, PBF shows large erythrocytes & reduced platelets & granulocytes. MCV is commonly increased. Reticulocytes are absent or few & lymphocyte numbers may be normal or reduced. No immature myeloid forms and abnormal platelets.

Question 20

Which of the following is not a feature of bone marrow cytology in aplastic anemia ?

A	Dilute aspirate
B	Hematopoietic cells occupying < 50 % of marrow
C	Megakaryocytes greatly reduced / absent
D	Mild megaloblastic erythropoiesis

Question 20 Explanation:

In AA, bone marrow is readily aspirated but dilute on smear. BM biopsy shows mainly fat under the microscope, with hematopoietic cells occupying <25% of marrow space

Question 21

Which of the following is false ?

A	Aplastic anemia is a disease of the young
B	Agranulocytosis is more frequent in elderly & women
C	In single lineage failure syndromes, progression to pancytopenia or leukemia is unusual
D	None of the above

Question 22

Severe aplastic anemia is defined by ?

A	Absolute neutrophil count < 500 / µL
B	Platelet count < 20,000 / µL
C	Absolute reticulocyte count < 60,000 / µL
D	All of the above

Question 22 Explanation:

Severe AA is defined by the presence of two of three parameters : absolute neutrophil count <500/µL, platelet count <20,000/µL & corrected reticulocyte count <1% (or absolute reticulocyte count <60,000/µL). Very severe disease is defined as absolute neutrophil count <200/µL

Question 23

Which of the following about aplastic anemia is false ?

A	CD34+ cells greatly reduced
B	Immunological deficiencies are common
C	Normal lymphocyte count
D	Complete recovery can occur with effective immunosuppressive therapy

Question 24

Which of the following have no value in treating severe acquired aplastic anemia ?

A	Hematopoietic growth factors (HGFs)
B	Glucocorticoids
C	Antithymocyte globulin (ATG)
D	Bone Marrow Transplantation

Question 24 Explanation:

Treatment options in severe acquired aplastic anemia are replacement of the absent hematopoietic cells by stem cell transplant, or suppression of immune system. Hematopoietic growth factors (HGFs) are not recommended as initial therapy for severe aplastic anemia, and even their roles as adjuncts to immunosuppression are not clear. Glucocorticoids are of no value

Question 25

Which of the following is used along with ALG or ATG to increase response rates in aplastic anemia ?

A	Azithromycin
B	Zinc
C	Cyclosporine
D	Vitamin E

Question 25 Explanation:

Addition of cyclosporine to ALG (antilymphocyte globulin) or ATG (antithymocyte globulin) increases response rates in AA.

Question 26

With standard regimen of ATG + cyclosporine for AA, improvement in granulocyte number is generally apparent within ?

A	1 month of treatment
B	2 months of treatment
C	3 months of treatment
D	4 months of treatment

Question 26 Explanation:

With standard regimen of ATG + cyclosporine for AA, improvement in granulocyte number is generally apparent within 2 months of treatment.

Question 27

In aplastic anemia treatment responders, MDS develops in what percentage of patients ?

A	10 %
B	15 %
C	20 %
D	25 %

Question 27 Explanation:

In aplastic anemia treatment responders, MDS develops in 15 % of patients.

Question 28

In adults, initial oral dose of cyclosporine in treatment of aplastic anemia is ?

A	2 mg/kg per day
B	8 mg/kg per day
C	12 mg/kg per day
D	20 mg/kg per day

Question 28 Explanation:

Cyclosporine is administered orally at an initial dose of 12 mg/kg per day in adults

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Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes – Part 1 | Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes – Part 2

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1. Which of the following is not a hypoproliferative anemia ?
A. Aplastic anemia
B. Myelodysplasia (MDS)
C. Pure red cell aplasia (PRCA)
D. Congenital dyserythropoietic anemia

2. Most cases of aplastic anemia are ?
A. Idiopathic
B. Due to drug exposure
C. Due to immune diseases
D. Due to inherited disorders

3. In aplastic anemia, age affected is ?
A. Children
B. Teens
C. Young adults
D. Teens & twenties and older adults

4. Which of the following is not a late effect of irradiation ?
A. Aplastic anemia
B. MDS
C. Leukemia
D. All of the above

5. Which of the following has consistent association with aplastic anemia ?
A. Benzene
B. Chloramphenicol
C. Carbamazapine
D. All of the above

6. Which of the following has consistent association with aplastic anemia ?
A. Gold
B. Phenylbutazone
C. Cimetidine
D. All of the above

7. Which of the following hepatitis virus infections most often precede posthepatitis marrow failure ?
A. A
B. B
C. C
D. None of the above

7. Cause of transient aplastic crisis in hemolytic anemias is ?
A. Hepatitis
B. Infectious mononucleosis
C. Parvovirus B19
D. All of the above

8. Aplastic anemia is strongly associated with which of the following collagen vascular syndrome ?
A. Polyarteritis nodosa
B. Schnitzler’s syndrome
C. Wegener’s granulomatosis
D. Eosinophilic fasciitis

9. Aplastic anemia is strongly associated with which of the following ?
A. Transfusion-associated graft-versus-host disease (GVHD)
B. Eosinophilic fasciitis
C. Systemic lupus erythematosus (SLE)
D. All of the above

10. Aplastic anemia is related to ?
A. Fanconi’s anemia

B. Paroxysmal nocturnal hemoglobinuria
C. Dyskeratosis congenita
D. All of the above

11. Which of the following about Fanconi’s anemia is false ?
A. Autosomal dominant disorder
B. Short stature, café au lait spots
C. Type A is due to mutation in FANCA
D. Increased risk of malignancy

12. Dyskeratosis congenita is characterized by all except ?
A. Abnormal skin pigmentation
B. Pancreatic insufficiency
C. Nail dystrophy
D. Mucosal leucoplakia

13. Dyskeratosis is due to mutations in which of the following genes ?
A. DKC1
B. TERC
C. TERT
D. Any of the above

14. Shwachman-Diamond syndrome features include all except ?
A. Pancreatic insufficiency
B. Malabsorption
C. Eosinophilia
D. Risk of aplastic anemia

15. Which cell in the bone marrow is helpful in differentiating aplastic anaemia from hypoplastic myelodysplastic syndromes ?
A. CD34 positive stem cells
B. CD38 positive stem cells
C. CD42 positive stem cells
D. CD46 positive stem cells

16. Which of the following is the most common early symptom in aplastic anemia ?
A. Bleeding
B. Infection
C. Weight loss
D. Jaundice

17. Which of the following is unusual in aplastic anemia ?
A. Infection on presentation
B. Lymphadenopathy
C. Splenomegaly
D. All of the above

18. Which of the following is not a feature of blood smear in aplastic anemia ?
A. Decreased mean corpuscular volume (MCV)
B. Few or absent reticulocytes
C. Normal lymphocyte number
D. Reduced platelets and granulocytes

19. Which of the following is not a feature of bone marrow cytology in aplastic anemia ?
A. Dilute aspirate
B. Hematopoietic cells occupying < 50 % of marrow
C. Megakaryocytes greatly reduced / absent
D. Mild megaloblastic erythropoiesis

20. Which of the following is false ?
A. Aplastic anemia is a disease of the young
B. Agranulocytosis is more frequent in elderly & women
C. In single lineage failure syndromes, progression to pancytopenia or leukemia is unusual
D. None of the above

21. Severe aplastic anemia is defined by ?
A. Absolute neutrophil count < 500 / µL
B. Platelet count < 20,000 / µL
C. Absolute reticulocyte count < 60,000 / µL

D. All of the above

22. Which of the following about aplastic anemia is false ?
A. CD34+ cells greatly reduced
B. Immunological deficiencies are common
C. Normal lymphocyte count
D. Complete recovery can occur with effective immunosuppressive therapy

23. Which of the following have no value in treating severe acquired aplastic anemia ?
A. Hematopoietic growth factors (HGFs)
B. Glucocorticoids
C. Antithymocyte globulin (ATG)
D. Bone Marrow Transplantation

24. Which of the following is used along with ALG or ATG to increase response rates in aplastic anemia ?
A. Azithromycin
B. Zinc
C. Cyclosporine
D. Vitamin E

25. With standard regimen of ATG + cyclosporine for AA, improvement in granulocyte number is generally apparent within ?
A. 1 month of treatment
B. 2 months of treatment
C. 3 months of treatment
D. 4 months of treatment

26. In aplastic anemia treatment responders, MDS develops in what percentage of patients ?
A. 10 %
B. 15 %
C. 20 %
D. 25 %

27. 1 In adults, initial oral dose of cyclosporine in treatment of aplastic anemia is ?

A. 2 mg/kg per day
B. 8 mg/kg per day
C. 12 mg/kg per day
D. 20 mg/kg per day

[MCQs] Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes – Part 1

I. Start the exam by click the “Start” button

Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes- Part 1

II. Preview all questions below

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