Deck 16: Hypoproliferative Anemias

Question 1

Aplastic anemia is most often caused by:

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

Answer 1

Unknown causes

Question 2

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

Answer 2

Diamond-Blackfan anemia

Question 3

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

Answer 3

Folate deficiency

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

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 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

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

A) Granulocyte count > 0.5 × 10⁹/L
B) Platelet count < 20 × 10⁹/L
C) Anemia
D) Anemia with corrected reticulocyte count >1%

Question 8

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 9

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 10

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

A) Longer life span of some red cells
B) Diminished p-glycoprotein
C) Autoimmune disorders
D) Lack of exposure

Question 11

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 12

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 13

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 14

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 15

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 16

Fanconi's syndrome is an example of:

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

Question 17

Which of the following puts a patient with long term exposure to benzene at risk for developing aplastic anemia?

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

Question 18

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

Question 19

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 20

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

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

Question 21

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 22

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

Question 23

List two examples of a congenital aplastic anemia.

Question 24

What congenital defects are found in Fanconi's syndrome?

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

Question 25

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 26

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) Pallor
B) Splenomegaly
C) Mucosal hemorrhage
D) Frequent infections

Question 27

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 28

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

Question 29

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

A) Granulocyte count 0.3 × 10⁹/L
B) Platelet count 5 × 10⁹/L
C) Anemia with absolute reticulocyte count of 22 × 10⁹/L
D) BM cellularity of 32%

Question 30

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 31

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