Deck 33: Myeloproliferative Neoplasms

Question 1

All of the following are included in the chronic myeloproliferative disorders except

A)acute myeloid leukemia.
B)chronic myelogenous leukemia.
C)polycythemia vera.
D)essential thrombocythemia.

Answer 1

acute myeloid leukemia.

Question 2

What clinical feature is more often associated with essential thrombocytosis than with the other chronic myeloproliferative disorders?

A)splenomegaly
B)bleeding and thrombosis
C)fatigue
D)infections

Answer 2

bleeding and thrombosis

Question 3

A patient has a normal white blood count,a moderately high platelet count,and mild anemia.The differential shows immature granulocytes,nucleated red cells,and teardrop-shaped red cells.Which of the following is most likely?

A)polycythemia vera
B)chronic myelogenous leukemia
C)primary myelofibrosis
D)essential thrombocythemia

Answer 3

primary myelofibrosis

Question 4

The Janus kinase (JAK)mutation is seen in more than 90% of cases of what disease?

A)chronic myelogenous leukemia
B)primary myelofibrosis
C)plasma cell myeloma
D)polycythemia vera

Question 5

The bone marrow fibrosis observed in primary myelofibrosis is due to

A)malignant proliferation of fibroblasts.
B)increased erythropoietin, which stimulates fibroblast proliferation.
C)increased release of fibroblastic growth factors such as platelet-derived growth factor.
D)decreased apoptosis of normal fibroblasts.

Question 6

Which chronic myeloproliferative disorder is treated with imatinib mesylate (Gleevec),a drug that inhibits tyrosine kinase?

A)polycythemia vera
B)chronic myelogenous leukemia
C)essential thrombocythemia
D)primary myelofibrosis

Question 7

Bone marrow or stem cell transplantation is the therapy of choice for patients younger than 55 years old with

A)essential thrombocytosis.
B)polycythemia vera.
C)chronic myelogenous leukemia.
D)all of the above.

Question 8

Which of the following would be an unexpected finding in chronic myelogenous leukemia?

A)increased eosinophils (EOs) and basophils in the peripheral blood
B)20% blasts in the peripheral blood
C)hypercellular bone marrow with granulopoiesis
D)white blood count of 100 * 109/L

Question 9

A patient has a platelet count of 1200 * 10⁹/L.Many platelets are giant and have abnormal shapes.A mild anemia is present.The bone marrow has increased megakaryocytes in clusters;iron stores are present.Which of the following is most likely?

A)essential thrombocythemia
B)chronic myelogenous leukemia
C)thrombocytosis secondary to blood loss
D)polycythemia vera

Question 10

The Philadelphia chromosome

A)is a balanced reciprocal translocation between chromosomes 7 and 19.
B)results in a chimeric gene (bcr/abl).
C)is diagnostic for polycythemia vera.
D)manifests as a tyrosine kinase that blocks cell maturation.

Question 11

Which of the following is an expected finding in polycythemia vera?

A)thrombocytosis
B)anemia
C)low leukocyte alkaline phosphatase
D)normal white count

Question 12

Chronic myelogenous leukemia often progresses to

A)chronic neutrophilic leukemia.
B)chronic lymphocytic leukemia.
C)acute leukemia, either myeloid or lymphoblastic.
D)chronic idiopathic myelofibrosis.

Question 13

What do all chronic myeloproliferative disorders share?

A)Philadelphia chromosome
B)increased red cell mass
C)increased blood cells; overlapping clinical and laboratory features
D)serious thromboembolic complications

Question 14

Which of the following is the most specific laboratory test to diagnose chronic myelogenous leukemia?

A)increased bone marrow fibrosis and reticulin fibers
B)increased EOs and basophils in peripheral blood
C)5% blasts in the bone marrow
D)leukocyte alkaline phosphatase

Question 15

Which of the following is true for chronic myelogenous leukemia?

A)It is a clonal process arising from the pluripotent stem cell.
B)It is most commonly found in children 5 to 10 years old.
C)Common symptoms include an elevated red cell mass.
D)Lymphadenopathy is common.

Question 16

Which of the following would be an unexpected finding in primary myelofibrosis?

A)80% blasts in the peripheral blood
B)micromegakaryocytes in the peripheral blood
C)autoantibodies
D)extramedullary hematopoiesis

Question 17

Which of the following is true for the mutated erythroid progenitors in polycythemia vera?

A)They require extremely high levels of erythropoietin for growth in vitro.
B)They are more resistant to apoptosis than normal erythroid progenitors.
C)They mature abnormally, giving rise to a marked increase in poikilocytosis in peripheral blood.
D)They are Philadelphia chromosome positive.

Question 18

A patient has an increased red count,hemoglobin,and hematocrit.Which of the following features points to secondary polycythemia over polycythemia vera?

A)increased white count
B)increased red cell mass
C)bone marrow erythroid hyperplasia
D)decreased arterial oxygen saturation

Question 19

A high red count,hemoglobin,and hematocrit are found in all of the following except

A)polycythemia vera.
B)bone marrow aplasia.
C)dehydration.
D)erythrocytosis secondary to hypoxia.

Question 20

Although found in most chronic myeloproliferative disorders,increased fibrosis detected by the silver techniques and trichrome stain is the key feature of

A)secondary polycythemia.
B)chronic myelogenous leukemia.
C)essential thrombocythemia.
D)primary myelofibrosis.