CHAPTER 33: MYELOPROLIFERATIVE NEOPLASMS

Question 1

(1) A peripheral blood film that shows increased neutrophils, basophils, eosinophils, and platelets is highly suggestive of:

a. AML
b. CML
c. MDS
d. Multiple myeloma

Answer 1

b. CML

Question 2

(2) Which of the following chromosome abnormalities is associated with CML?

a. t(15;17)
b. t(8;14)
c. t(9;22)
d. Monosomy 7

Answer 2

c. t(9;22)

Question 3

(3) A patient has a WBC count of 30 x 10^9/L and the following WBC differential:

Segmented neutrophils—38%
Bands—17%
Metamyelocytes—7%
Myelocytes—20%
Promyelocytes—10%
Eosinophils—3%
Basophils—5%

Which of the following test results would be helpful in determining whether the patient has CML?

a. Nitroblue tetrazolium reduction product increased
b. Myeloperoxidase increased
c. Periodic acid–Schiff staining decreased
d. FISH positive for BCR/ABL1 fusion

Answer 3

d. FISH positive for BCR/ABL1 fusion

Question 4

(4) A patient in whom CML has previously been diagnosed has circulating blasts and promyelocytes that total 30% of leukocytes. The disease is considered to be in what phase?

a. Chronic stable phase
b. Accelerated phase
c. Transformation to acute leukemia
d. Temporary remission

Answer 4

c. Transformation to acute leukemia

Question 5

(5) The most common mutation found in patients with primary PV is:

a. BCR/ABL
b. Philadelphia chromosome
c. JAK2 V617F
d. t(15;17)

Answer 5

c. JAK2 V617F

Question 6

(6) The peripheral blood in PV typically manifests:

a. Erythrocytosis only
b. Erythrocytosis and thrombocytosis
c. Erythrocytosis, thrombocytosis, and granulocytosis
d. Anemia and thrombocytopenia

Answer 6

c. Erythrocytosis, thrombocytosis, and granulocytosis

Question 7

(7) A patient has a platelet count of 700 x 10^9/L with abnormalities in the size, shape, and granularity of platelets; a WBC count of 12 x10^9/L; and hemoglobin of 11 g/dL. The Philadelphia chromosome is not present. The most likely diagnosis is:

a. PV
b. ET
c. CML
d. Leukemoid reaction

Answer 7

b. ET

Question 8

(8) Complications of ET include all of the following except:

a. Thrombosis
b. Hemorrhage
c. Seizures
d. Infections

Answer 8

d. Infections

Question 9

(9) Which of the following patterns is characteristic of the peripheral blood in patients with PMF?

a. Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes
b. Abnormal platelets only
c. Hypochromic erythrocytes, immature granulocytes, and normal platelets
d. Spherocytes, immature granulocytes, and increased numbers of platelets

Answer 9

a. Teardrop-shaped erythrocytes, nucleated RBCs, immature granulocytes

Question 10

(10) The myelofibrosis associated with PMF is a result of:

a. Apoptosis resistance in the fibroblasts of the bone marrow
b. Impaired production of normal collagenase by the mutated cells
c. Enhanced activity of fibroblasts owing to increased stimulatory cytokines
d. Increased numbers of fibroblasts owing to cytokine stimulation of the pluripotential stem cells

Answer 10

c. Enhanced activity of fibroblasts owing to increased stimulatory cytokines