Bone Marrow Failure

Question 1

1. The clinical consequences of pancytopenia include:
   a. Pallor and thrombosis
   b. Kidney failure and fever
   c. Fatigue, infection, and bleeding
   d. Weakness, hemolysis, and infection

Answer 1

c. Fatigue, infection, and bleeding

Question 2

2. Idiopathic acquired aplastic anemia is due to a(n):
   a. Drug reaction
   b. Benzene exposure
   c. Inherited mutation in stem cells
   d. Unknown cause

Answer 2

d. Unknown cause

Question 3

3. The pathophysiologic mechanism in acquired idiosyncratic
   aplastic anemia is:
   a. Replacement of bone marrow by abnormal cells
   b. Destruction of stem cells by autoimmune T cells
   c. Defective production of hematopoietic growth factors
   d. Inability of bone marrow stroma to support stem cells

Answer 3

b. Destruction of stem cells by autoimmune T cells

Question 4

4. Based on the criteria in Table 22-1, what is the aplastic anemia
   classification of a 15-year-old female with a bone marrow
   cellularity of 10%, hemoglobin of 7 g/dL, absolute neutrophil
   count of 0.1 3 109/L, and platelet count of 10 3 109/L?
   a. Nonsevere
   b. Moderate
   c. Severe
   d. Very severe

Answer 4

d. Very severe

Question 5

5. The most consistent peripheral blood findings in severe
   aplastic anemia are:
   a. Hairy cells, monocytopenia, and neutropenia
   b. Macrocytosis, thrombocytopenia, and neutropenia
   c. Blasts, immature granulocytes, and thrombocytopenia
   d. Polychromasia, nucleated RBCs, and hypersegmented
   neutrophils

Answer 5

b. Macrocytosis, thrombocytopenia, and neutropenia

Question 6

6. The treatment that has shown the best success rate in young
   patients with severe aplastic anemia is:
   a. Immunosuppressive therapy
   b. Long-term red blood cell and platelet transfusions
   c. Administration of hematopoietic growth factors and
   androgens
   d. Stem cell transplant with an HLA-identical sibling

Answer 6

d. Stem cell transplant with an HLA-identical sibling

Question 7

7. The test that is most useful in differentiating FA from other
   causes of pancytopenia is:
   a. Bone marrow biopsy
   b. Ham acidified serum test
   c. Diepoxybutane-induced chromosome breakage
   d. Flow cytometric analysis of CD55 and CD59 cells

Answer 7

c. Diepoxybutane-induced chromosome breakage

Question 8

8. Mutations in genes that code for the telomerase complex
   may induce bone marrow failure by causing which one of
   the following?
   a. Resistance of stem cells to normal apoptosis
   b. Autoimmune reaction against telomeres in stem cells
   c. Decreased production of hematopoietic growth factors
   d. Premature death of hematopoietic stem cells

Answer 8

d. Premature death of hematopoietic stem cells

Question 9

9. Diamond-Blackfan anemia differs from inherited aplastic
   anemia in that in the former:
   a. Reticulocyte count is increased
   b. Fetal hemoglobin is decreased
   c. Only erythropoiesis is affected
   d. Congenital malformations are absent

Answer 9

c. Only erythropoiesis is affected

Question 10

10. Which anemia should be suspected in a patient with refractory anemia, reticulocytopenia, hemosiderosis, and
    binucleated erythrocyte precursors in the bone marrow?
    a. Fanconi anemia
    b. Dyskeratosis congenita
    c. Acquired aplastic anemia
    d. Congenital dyserythropoietic anemia

Answer 10

d. Congenital dyserythropoietic anemia

Question 11

11. The primary pathophysiologic mechanism of anemia associated with chronic kidney disease is:
    a. Inadequate production of erythropoietin
    b. Excessive hemolysis
    c. Hematopoietic stem cell mutation
    d. Toxic destruction of stem cells

Answer 11

a. Inadequate production of erythropoietin