Deck 16: Hypoproliferative Anemias

Question 1

Fanconi's syndrome is an example of:

A)Constitutional aplastic anemia
B)Acquired aplastic anemia
C)Constitutional anemia from external stimuli
D)Acquired aplastic anemia from external exposure

Answer 1

Constitutional aplastic anemia

Question 2

Which of the following puts a patient taking chloramphenicol at risk for developing aplastic anemia?

A)The drug promotes an immune response against stem cells.
B)The drug is toxic to the bone marrow.
C)The drug results in stem cell resistance to essential cytokines.
D)The drug causes decreased cytokine production.

Answer 2

The drug is toxic to the bone marrow.

Question 3

A 55-year-old male is undergoing dialysis to treat his renal failure.Routine blood examination shows a low RBC count,low H&H,and a normal MCV.What is the most important factor contributing to this anemia?

A)Ineffective erythropoiesis
B)Hemolysis
C)Decreased erythropoietin production
D)Presence of cytotoxic antibodies to erythropoietin-sensitive cells

Answer 3

Decreased erythropoietin production

Question 4

Which of the following treatments offers the best prognosis for aplastic anemia?

A)Synthetic cytokines
B)Bone marrow transplant
C)Immunosuppressive therapy
D)Stem cell transplant

Question 5

Aplastic anemia is most often caused by:

A)Drugs
B)Radiation
C)Unknown causes
D)Infectious agents

Question 6

Hypoproliferative anemia is defined as:

A)Decreased hematopoiesis in the bone marrow
B)Decreased hematopoiesis in the liver
C)Decreased erythropoiesis in the bone marrow
D)Ineffective erythropoiesis in the bone marrow

Question 7

What CBC parameter would help differentiate Fanconi's syndrome from Diamond-Blackfan anemia?

A)MCV
B)WBC and PLT counts
C)RBC counts
D)Differential

Question 8

A 5-year-old boy with malformed thumbs and microcephaly has been suffering from prolonged bleeding episodes,extreme fatigue,and persistent repetitive infections.Cytogenetic analysis shows increased chromosome breakage with the addition of diepoxybutane.Based on these findings,what is the patient most likely suffering from?

A)CDA II
B)Pure red cell aplasia
C)Diamond-Blackfan anemia
D)Fanconi's syndrome

Question 9

A 3-month-old infant is exhibiting pallor and fatigue.The pediatrician orders a CBC,which displays a low RBC count,H&H,and normal WBC and PLT counts.EPO levels are increased.BM aspirate shows prominent erythroid hypoplasia.Cytogenetic analysis reveals the presence of RPS19 mutation.Based on these findings,what is the patient most likely suffering from?

A)CDA II
B)Pure red cell aplasia
C)Diamond-Blackfan anemia
D)Fanconi's syndrome

Question 10

How can infection with the Epstein-Barr virus (EBV)lead to aplastic anemia?

A)EBV causes defects in precursor cells.
B)EBV infects stem cells,and an immune response to destroy the stem cells is initiated.
C)EBV creates immune complexes on the surface of the stem cell that damages it.
D)Infection with the virus shuts down cytokine production by macrophages.

Question 11

Diamond-Blackfan syndrome anemia is a rare congenital progressive erythrocyte aplasia.What is the most probable defect causing this rare disorder?

A)Deficiency of erythropoietin
B)Antibodies against erythropoietin
C)Intrinsic defect of erythroid progenitor cells
D)Increased reticulocytes

Question 12

The presence of what poikilocyte would lead to a suspicion of myelophthisic anemia rather than pure red cell aplasia?

A)Dacryocyte
B)Drepanocyte
C)Schistocyte
D)Stomatocyte

Question 13

Patients with renal disease are at risk for developing megaloblastic anemia because of:

A)Decreased EPO production and utilization
B)Increased NADPH production
C)Folate deficiency
D)Acute blood loss

Question 14

Aplastic anemia in the acquired form can result from drugs or chemical agent exposure.If the link cannot be made to any environmental factor,what form of anemia does the client have?

A)Pure red cell aplasia
B)Anemia of chronic renal disease
C)Idiopathic
D)Chronic

Question 15

Which of the following describes the bone marrow in a patient with Fanconi's syndrome?

A)Hypercellular with erythroid hyperplasia
B)Hypocellular with normal M:E ratio
C)Normocellular with decreased M:E ratio
D)Hypocellular with elevated M:E ratio

Question 16

Which of the following tests would help differentiate TEC from DBA?

A)RBC count and RBC indices
B)Bone marrow cellularity and M:E ratio
C)Fetal erythrocyte characteristics
D)Ham test

Question 17

The cytopenias associated with MDS are caused by:

A)Dyshematopoiesis
B)Bone marrow replacement
C)Hypoplasia of the bone marrow
D)Decreased cytokine production

Question 18

Which of the following contributes to the production of hypocellular bone marrow?

A)Damage to the stem or progenitor cells in the bone marrow
B)Iron storage disease
C)Gene mutations
D)Hemoglobinopathy

Question 19

What is a viable explanation as to why some individuals suffer stem cell damage when exposed to chemical agents but others do not?

A)Longer life span of some red cells
B)Superior detoxification
C)Autoimmune disorders
D)Lack of exposure

Question 20

Which of the following is among the diagnostic criteria for aplastic anemia?

A)Granulocyte count > 0.5 x 10⁹/L
B)Platelet count < 20 × 10⁹/L
C)Anemia
D)Anemia with

Question 21

Explain how exposure to the following can lead to aplastic anemia.
a.Chloramphenicol
b.Benzene
c.Gamma radiation
d.Starvation

Question 22

Acquired chronic pure red cell aplasia is a rare disorder encountered in association with several autoimmune disorders.What does the mechanism appear to be?

A)B cell-mediated immunosuppression of neutrophils
B)T cell-mediated immunosuppression of erythropoiesis
C)Cytokine-mediated immunosuppression of erythropoiesis
D)Increased serum erythropoietin

Question 23

Recent evidence suggests that the pathophysiology of most cases of acquired aplastic anemia is most likely:

A)Drug exposure
B)Viral infections
C)Defective stem cells
D)Immunologic suppression of hematopoiesis

Question 24

Which CDA is characterized by a positive Ham test and a negative sucrose lysis test?

A)CDA I
B)CDA II
C)CDA III
D)CDA IV

Question 25

Aplastic anemia is usually first noted by bleeding and the presence of petechial hemorrhages.What clinical sign in the early stages of the disease should cause doubt on the diagnosis of aplastic anemia?

A)Hepatosplenomegaly
B)Splenomegaly
C)Lymph node enlargement
D)Fever

Question 26

All of the following are considered diagnostic criteria for aplastic anemia except:

A)
B)
C)
D)

Question 27

Anemia in hypersplenism differs from anemia found in true hypoproliferative anemias because in hypersplenism,there is:

A)Reticulocytosis
B)Ineffective hematopoiesis
C)Dyshematopoiesis
D)Hypoplastic bone marrow

Question 28

Define constitutional aplastic anemia,and give an example.

Question 29

What congenital defects are found in Fanconi's syndrome?

A)Hypergonadism
B)Skin hypopigmentation
C)Mental retardation
D)Thrombocytic purpura

Question 30

Correlate the prognosis associated with the following treatments for aplastic anemia.
a.Bone marrow transplant
b.Cytokine therapy
c.Stem cell transplant
d.Immunosuppression therapy

Question 31

Chronic renal disease is a common cause of anemia.Which of the following is one of the possible causes of anemia in chronic renal disease?

A)Increased erythropoietin production
B)Increased erythrocyte survival
C)Blood loss
D)Iron production

Question 32

Laboratory findings include a blood urea nitrogen >30 mg/dL,serum ferritin levels higher than normal,and a normocytic,normochromic morphology.What cause can be attributed to the anemia?

A)Chronic renal disease
B)Iron deficiency
C)Diamond-Blackfan anemia
D)Fanconi's syndrome