[MCQs] Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes - Part 2

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Question 1

Trough blood levels of cyclosporine in treatment of aplastic anemia should be between ?

A	150 and 200 ng / mL
B	250 and 400 ng / mL
C	450 and 600 ng / mL
D	650 and 800 ng / mL

Question 1 Explanation:

Trough cyclosporine blood levels in treatment of AA should be between 150 - 200 ng/mL

Question 2

Most important side effects of chronic cyclosporine treatment include all except ?

A	Nephrotoxicity
B	Hepatotoxicity
C	Hypertension
D	Seizures

Question 2 Explanation:

Most important side effects of chronic cyclosporine treatment are nephrotoxicity, hypertension, seizures & opportunistic infections.

Question 3

Dose of Horse ATG in treatment of aplastic anemia is ?

A	40 mg/kg per day for 1 day
B	40 mg/kg per day for 2 days
C	40 mg/kg per day for 3 days
D	40 mg/kg per day for 4 days

Question 3 Explanation:

Horse ATG is given at 40 mg/kg per day for 4 days.

Question 4

The single best method of preventing the spread of infection while treating patients of aplastic anemia is ?

A	Prompt institution of parenteral, broad-spectrum antibiotics
B	Hand washing
C	Nonabsorbed antibiotics for gut decontamination
D	Total reverse isolation

Question 4 Explanation:

Hand washing, the single best method of preventing the spread of infection, but remains a neglected practice.

Question 5

In chronic anemia, iron chelators are given after how many blood transfusions ?

A	10
B	20
C	40
D	50

Question 5 Explanation:

In chronic anemia, iron chelators (deferoxamine & deferasirox) are given at around the fiftieth transfusion to avoid secondary hemochromatosis.

Question 6

PRCA is characterized by ?

A	Anemia
B	Reticulocytopenia
C	Absent or rare erythroid precursor cells in bone marrow
D	All of the above

Question 6 Explanation:

PRCA is characterized by anemia, reticulocytopenia & absent or rare erythroid precursor cells in bone marrow.

Question 7

Congenital pure red cell aplasia is known as ?

A	Fanconi’s anemia
B	Shwachman-Diamond syndrome
C	Kostmann’s Syndrome
D	Diamond-Blackfan anemia

Question 7 Explanation:

Diamond-Blackfan anemia or congenital PRCA is diagnosed at birth or in early childhood & often responds to glucocorticoid treatment. A minority of patients have etiologic mutations in a ribosomal RNA processing gene called RPS19.

Question 8

Congenital pure red cell aplasia (Diamond-Blackfan syndrome) is which variety of PRCA ?

A	Fetal red blood cell aplasia
B	Hereditary pure red cell aplasia
C	Acquired pure red cell aplasia
D	Idiopathic

Question 9

Drug that may cause PRCA is ?

A	Phenytoin
B	Chloramphenicol
C	Isoniazid
D	All of the above

Question 9 Explanation:

Drug that may cause PRCA include phenytoin, azathioprine, chloramphenicol, procainamide, isoniazid and erythropoietin

Question 10

PRCA may be associated with which of the following ?

A	Thymoma
B	Chronic lymphocytic leukemia
C	Subcutaneous administration of erythropoietin
D	All of the above

Question 10 Explanation:

PRCA may be associated with thymoma, large granular lymphocytosis, chronic lymphocytic leukemia, hypogammaglobulinemic and subcutaneous administration of erythropoietin

Question 11

Pathognomonic cell in bone marrow of PRCA patients with B19 parvovirus infection is ?

A	Uninuclear megakaryocyte
B	Giant megakayroblast
C	Giant pronormoblast
D	Ringed sideroblast

Question 11 Explanation:

Bone marrow in PRCA due to chronic parvovirus infection shows red cell aplasia & giant pronormoblasts, which is the cytopathic sign of B19 parvovirus infection

Question 12

B19 parvovirus tropism for human erythroid progenitor cells is due to its use of which erythrocyte antigen ?

A	L
B	M
C	P
D	S

Question 12 Explanation:

B19 parvo viral tropism for human erythroid progenitor cells is due to its use of erythrocyte P antigen as a cellular receptor for entry

Question 13

PRCA patients with persistent B19 parvovirus infection respond best to ?

A	Glucocorticoids
B	Intravenous immunoglobulin therapy
C	Cyclosporine
D	Daclizumab

Question 13 Explanation:

Almost all patients with PRCA due to persistent B19 parvovirus infection respond to IV Ig therapy (0.4 gram/kg daily for 5 days). Majority of patients with idiopathic PRCA respond favorably to immunosuppression with glucocorticoids, cyclosporine, ATG, azathioprine, cyclophosphamide, and daclizumab (antibody to IL-2 receptor)

Question 14

Which of the following is a feature of myelodysplasias (MDS) ?

A	Cytopenias
B	Dysmorphic cellular bone marrow
C	Ineffective blood cell production
D	All of the above

Question 14 Explanation:

Myelodysplasias (MDS) are characterized by cytopenias, dysmorphic cellular bone marrow and by ineffective blood cell production

Question 15

Which of the following is a myelodysplastic syndrome ?

A	Refractory anemia (RA
B	Refractory anemia with ringed sideroblasts (RARS)
C	Refractory anemia with excess blasts (RAEB)
D	All of the above

Question 15 Explanation:

According to French-American-British Cooperative Group (1983), five entities of MDS are refractory anemia (RA), refractory anemia with ringed sideroblasts (RARS), refractory anemia with excess blasts (RAEB), refractory anemia with excess blasts in transformation (RAEB-t) and chronic myelomonocytic leukemia (CMML). World Health Organization (WHO) classification (2002) classified Myelodysplastic Syndromes/Neoplasms differently

Question 16

Which of the following is the most frequent classe in WHO estimated proportion of patients with MDS ?

A	Refractory anemia (RA)
B	Refractory anemia with ring sideroblasts (RARS)
C	Refractory cytopenias with multilineage dysplasia (RCMD)
D	Refractory anemia with excess blasts, Type 1 (RAEB-1)

Question 17

Which of the following is false about myelodysplastic syndrome ?

A	Idiopathic MDS is a disease of elderly
B	Clonal hematopoietic stem cell disorder
C	Apoptosis of marrow cells increased
D	None of the above

Question 17 Explanation:

Idiopathic MDS is a disease of the elderly (mean age at onset - 70 years) and.is rare in children. Therapy-related MDS is not age-related. It is a clonal hematopoietic stem cell disorder leading to impaired cell proliferation and differentiation. Apoptosis of marrow cells is increased in MDS

Question 18

In secondary MDS, latent period is least following which of the following cancer treatments ?

A	DNA topoisomerase inhibitors
B	Busulfan
C	Nitrosourea
D	Procarbazine

Question 18 Explanation:

Secondary MDS occurs as a late toxicity of cancer treatment. With busulfan, nitrosourea, or procarbazine, the latent period is 5 - 7 years and with DNA topoisomerase inhibitors it is 2 years

Question 19

Which of the following skin conditions is related to MDS ?

A	Gardner syndrome
B	Sweet’s syndrome
C	Cowden disease
D	Torre syndrome

Question 19 Explanation:

Unusual skin lesions, including Sweet’s syndrome (febrile neutrophilic dermatosis), occur with MDS, hematologic malignancies, solid tumors, or inflammatory bowel disease

Question 20

Sideroblasts have granules consisting of ?

A	Ferritin
B	Transferrin
C	Glycogen
D	All of the above

Question 21

Sideroblasts are ?

A	Developing erythroblasts
B	Developing myeloblasts
C	Defective erythroblasts
D	Defective myeloblasts

Question 22

Normal percentage of sideroblasts in bone marrow is ?

A	5 %
B	10 %
C	40 %
D	75 %

Question 22 Explanation:

Storage iron is in the form of ferritin or hemosiderin. In bone marrow smears, small ferritin granules are normally seen under oil immersion in 20 - 40% of developing erythroblasts. Such cells are called sideroblasts.

Question 23

In ‘ringed sideroblasts’, the accumulation of iron is around ?

A	In ‘ringed sideroblasts’, the accumulation of iron is around ?
B	Nucleus
C	Mitochondria
D	Endoplasmic reticulum

Question 24

Sideroblastic anemia usually points to the diagnosis of ?

A	Aplastic anemia
B	Myelodysplasia
C	Pernicious anemia
D	All of the above

Question 25

Bone marrow in myelodysplasia may show all except ?

A	Normal or hypercellularity
B	Ringed sideroblasts
C	Hypogranulation & hyposegmentation in granulocytic precursors
D	Decrease in myeloblasts

Question 25 Explanation:

No single characteristic feature of marrow morphology distinguishes MDS. BM is usually normal or hypercellular with ringed sideroblasts in erythroid lineage, hypogranulation & hyposegmentation in granulocytic precursors, increase in myeloblasts and reduced number of megakaryocytes with disorganized nuclei

Question 26

In MDS, prognosis strongly correlates with ?

A	Hypogranulation in granulocytic precursors
B	Ringed sideroblasts
C	Proportion of marrow blasts
D	Circulating myeloblasts

Question 26 Explanation:

Prognosis strongly correlates with the proportion of marrow blasts. Circulating myeloblasts usually correlate with marrow blast numbers.

Question 27

Which of the following drugs has a role in treatment of MDS ?

A	Which of the following drugs has a role in treatment of MDS ?
B	Amifostine
C	Lenalidomide
D	All of the above

Question 27 Explanation:

Only stem cell transplantation offers cure in MDS. Azacitidine (75 mg/m2 daily s/c for 7 days), Decitabine (15 mg/m2 IV infusion, TDS for three days), Lenalidomide (10 mg orally daily for 3 months), Amifostine (blocks apoptosis), G-CSF + Erythropoietin have a role in MDS. ATG and antiCD52 monoclonal antibody Campath is effective in younger MDS patients who bear the histocompatability antigen HLA-DR15

Question 28

Which of the following is false about myelophthisis ?

A	Secondary myelofibrosis
B	Leukoerythroblastic blood smear
C	Ineffective erythropoiesis
D	None of the above

Question 28 Explanation:

Myelophthisis or secondary myelofibrosis is a reactive phenomenon. Fibrosis of BM occurs as a response to invading tumor cells, infections (Mycobacterium, fungi, HIV), sarcoidosis, Gaucher disease, congenital osteopetrosis, radiation therapy or treatment with radiomimetic drug

Question 29

Which of the following is false about myelophthisis ?

A	Myeloid metaplasia
B	Pancytopenia
C	Increased circulating hematopoietic progenitor cells
D	None of the above

Question 29 Explanation:

Pathophysiology of myelophthisis has three distinct features: proliferation of fibroblasts in marrow space (myelofibrosis), myeloid metaplasia i.e. extension of hematopoiesis into long bones and into extramedullary sites (spleen, liver, and lymph nodes) and ineffective erythropoiesis. Pancytopenia is seen despite very large numbers of circulating hematopoietic progenitor cells.

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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. Trough blood levels of cyclosporine in treatment of aplastic anemia should be between ?
A. 150 and 200 ng / mL
B. 250 and 400 ng / mL
C. 450 and 600 ng / mL
D. 650 and 800 ng / mL

2. Most important side effects of chronic cyclosporine treatment include all except ?
A. Nephrotoxicity
B. Hepatotoxicity
C. Hypertension
D. Seizures

3. Dose of Horse ATG in treatment of aplastic anemia is ?
A. 40 mg/kg per day for 1 day
B. 40 mg/kg per day for 2 days
C. 40 mg/kg per day for 3 days
D. 40 mg/kg per day for 4 days

4. The single best method of preventing the spread of infection while treating patients of aplastic anemia is ?
A. Prompt institution of parenteral, broad-spectrum antibiotics
B. Hand washing
C. Nonabsorbed antibiotics for gut decontamination
D. Total reverse isolation

5. In chronic anemia, iron chelators are given after how many blood transfusions ?
A. 10
B. 20
C. 40
D. 50

6. PRCA is characterized by ?

A. Anemia
B. Reticulocytopenia
C. Absent or rare erythroid precursor cells in bone marrow
D. All of the above

7. Congenital pure red cell aplasia is known as ?
A. Fanconi’s anemia
B. Shwachman-Diamond syndrome
C. Kostmann’s Syndrome
D. Diamond-Blackfan anemia

8. Congenital pure red cell aplasia (Diamond-Blackfan syndrome) is which variety of PRCA ?
A. Fetal red blood cell aplasia
B. Hereditary pure red cell aplasia
C. Acquired pure red cell aplasia
D. Idiopathic

9. Drug that may cause PRCA is ?
A. Phenytoin
B. Chloramphenicol
C. Isoniazid
D. All of the above

10. PRCA may be associated with which of the following ?
A. Thymoma
B. Chronic lymphocytic leukemia
C. Subcutaneous administration of erythropoietin
D. All of the above

11. Pathognomonic cell in bone marrow of PRCA patients with B19 parvovirus infection is ?
A. Uninuclear megakaryocyte
B. Giant megakayroblast
C. Giant pronormoblast
D. Ringed sideroblast

12. B19 parvovirus tropism for human erythroid progenitor cells is due to its use of which erythrocyte antigen ?

A. L
B. M

C. P
D. S

13. PRCA patients with persistent B19 parvovirus infection respond best to ?
A. Glucocorticoids
B. Intravenous immunoglobulin therapy
C. Cyclosporine
D. Daclizumab

14. Which of the following is a feature of myelodysplasias (MDS) ?
A. Cytopenias
B. Dysmorphic cellular bone marrow
C. Ineffective blood cell production
D. All of the above

15. Which of the following is a myelodysplastic syndrome ?
A. Refractory anemia (RA)
B. Refractory anemia with ringed sideroblasts (RARS)
C. Refractory anemia with excess blasts (RAEB)
D. All of the above

16. Which of the following is the most frequent classe in WHO estimated proportion of patients with MDS ?
A. Refractory anemia (RA)
B. Refractory anemia with ring sideroblasts (RARS)
C. Refractory cytopenias with multilineage dysplasia (RCMD)
D. Refractory anemia with excess blasts, Type 1 (RAEB-1)

17. Which of the following is false about myelodysplastic syndrome ?
A. Idiopathic MDS is a disease of elderly
B. Clonal hematopoietic stem cell disorder
C. Apoptosis of marrow cells increased
D. None of the above

18. In secondary MDS, latent period is least following which of the following cancer treatments ?
A. DNA topoisomerase inhibitors
B. Busulfan
C. Nitrosourea
D. Procarbazine

19. Which of the following skin conditions is related to MDS ?
A. Gardner syndrome
B. Sweet’s syndrome
C. Cowden disease
D. Torre syndrome

20. Sideroblasts have granules consisting of ?
A. Ferritin
B. Transferrin
C. Glycogen
D. All of the above

21. Sideroblasts are ?
A. Developing erythroblasts
B. Developing myeloblasts
C. Defective erythroblasts
D. Defective myeloblasts

22. Normal percentage of sideroblasts in bone marrow is ?
A. 5 %
B. 10 %
C. 40 %
D. 75 %

23. In ‘ringed sideroblasts’, the accumulation of iron is around ?
A. Cell membrane
B. Nucleus
C. Mitochondria
D. Endoplasmic reticulum

24. Sideroblastic anemia usually points to the diagnosis of ?
A. Aplastic anemia
B. Myelodysplasia
C. Pernicious anemia
D. All of the above

25. In secondary MDS, latent period is least following which of the following cancer treatments ?
A. DNA topoisomerase inhibitors
B. Busulfan
C. Nitrosourea
D. Procarbazine

26. Which of the following skin conditions is related to MDS ?
A. Gardner syndrome
B. Sweet’s syndrome
C. Cowden disease
D. Torre syndrome

27. Sideroblasts have granules consisting of ?
A. Ferritin
B. Transferrin
C. Glycogen
D. All of the above

28. Sideroblasts are ?
A. Developing erythroblasts
B. Developing myeloblasts
C. Defective erythroblasts
D. Defective myeloblasts

29. Normal percentage of sideroblasts in bone marrow is ?
A. 5 %
B. 10 %
C. 40 %
D. 75 %

30. In ‘ringed sideroblasts’, the accumulation of iron is around ?
A. Cell membrane
B. Nucleus
C. Mitochondria
D. Endoplasmic reticulum

31. Sideroblastic anemia usually points to the diagnosis of ?
A. Aplastic anemia
B. Myelodysplasia
C. Pernicious anemia
D. All of the above

[MCQs] Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes – Part 2

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Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes- Part 2

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