Question 1

Almost all patients with this CMPD are positive for the JAK2(V617F) mutation:

Accepted Answer

A)  CIMF 
B)  ET 
C)  PV 
D)  CML 
E)  None of the above

Question 2

The myeloproliferative disorder typically associated with the highest platelet count [>1 million/L (1000 F1F1F1S1F1F1F10 109/L)] is:

Accepted Answer

A)  Essential thrombocythemia 
B)  Polycythemia rubra vera 
C)  CML in chronic phase 
D)  Idiopathic myelofibrosis 
E)  CML in blast transformation

Question 3

In the hypercellular (prefibrotic) phase of chronic idiopathic myelofibrosis (CIMF), the bone marrow displays panhyperplasia with a predominance of __________.

Accepted Answer

A)  Megakaryocytes 
B)  Granulocytes 
C)  Lymphocytes 
D)  Erythrocytes 
E)  A and B are correct

Question 4

A patient diagnosed with myelofibrosis secondary to gastrointestinal disease may develop a __________ anemia due to excessive blood loss.

Accepted Answer

A)  Normocytic/normochromic 
B)  Microcytic/hypochromic 
C)  Macrocytic/hyperchromic 
D)  Normocytic/hypochromic 
E)  None of the above

Question 5

What is the hallmark physical finding in CMPDs?

Accepted Answer

A)  Splenomegaly 
B)  Ecchymosis 
C)  Plasmacytoma 
D)  Back pain 
E)  Headaches

Question 6

How is essential thrombocythemia differentiated from chronic myelocytic leukemia?

Accepted Answer

A)  Presence of bone marrow fibrosis 
B)  Absence of Philadelphia chromosome 
C)  Presence of nucleated red blood cells (NRBCs) 
D)  Thrombocytosis 
E)  All of the above

Question 7

Which clinical manifestations in polycythemia vera are consistent with those of other myeloproliferative syndromes?

Accepted Answer

A)  Splenomegaly 
B)  Myeloid metaplasia 
C)  Myelofibrosis 
D)  All of the above 
E)  None of the above

Question 8

Metabolic consequences of myelofibrosis include all of the following except:

Accepted Answer

A)  Night sweats 
B)  Hyperuricemia 
C)  Weight loss 
D)  Hypotension 
E)  Anorexia

Question 9

When essential thrombocythemia leads to iron deficiency anemia from associated bleeding, a __________ anemia will result.

Accepted Answer

A)  Normocytic/normochromic 
B)  Microcytic/hypochromic 
C)  Normocytic/hypochromic 
D)  Macrocytic/normochromic 
E)  None of the above

Question 10

In polycythemia vera, therapeutic phlebotomies reduce the total red cell mass by removing 350-500 mL over 2-3 days until the hematocrit is maintained at __________ percent.

Accepted Answer

A)  50-60 
B)  40-45 
C)  30-40 
D)  >60 
E)  <30

Question 11

A __________ anemia is found in most myelofibrosis patients at presentation:

Accepted Answer

A)  Normocytic/hypochromic 
B)  Normocytic/normochromic 
C)  Macrocytic/hyperchromic 
D)  Microcytic/hypochromic 
E)  None of the above

Question 12

Which is the expected platelet count in essential thrombocythemia?

Accepted Answer

A)  600-2500 F1F1F1S1F1F1F10 109/L 
B)  150-400 F1F1F1S1F1F1F10 109/L 
C)  500-750 F1F1F1S1F1F1F10 109/L 
D)  50-300 F1F1F1S1F1F1F10 109/L 
E)  None of the above

Question 13

Hemorrhage and thrombosis are physiologic complications of __________ due to excessive proliferation of the megakaryocyte lineage.

Accepted Answer

A)  Thrombocythemia 
B)  Thrombocytopenia 
C)  Hyperuricemia 
D)  Hypercalcemia 
E)  All of the above

Question 14

Tissue hypoxia and increased erythropoietic activity is an example of ___________ erythrocytosis.

Accepted Answer

A)  Relative 
B)  Stress 
C)  Secondary 
D)  Primary 
E)  None of the above

Question 15

What is the usefulness of myelosuppression as a treatment in polycythemia vera?

Accepted Answer

A)  Alleviates pruritus 
B)  Suppresses abnormal bone marrow proliferation 
C)  Controls effects of splenomegaly 
D)  Suppresses thrombocytosis 
E)  All of the above

Question 16

Which of the following are causes of reactive thrombocytosis?

Accepted Answer

A)  Acute hemorrhage 
B)  Postsplenectomy and hyposplenism 
C)  Malignancy 
D)  Iron-deficiency anemia 
E)  All of the above

Question 17

In the absolute erythrocytosis, the red cell mass is __________.

Accepted Answer

A)  Decreased 
B)  Increased 
C)  Normal 
D)  All of the above 
E)  None of the above

Question 18

In secondary erythrocytosis, the underlying mechanism is release of __________ as part of a compensatory effect to minimize impending tissue hypoxia.

Accepted Answer

A)  Erythropoietin 
B)  Thrombopoietin 
C)  Interleukin-1 
D)  Colony-stimulating factors 
E)  None of the above

Question 19

In both absolute erythrocytosis and relative erythrocytosis the hematocrit is __________.

Accepted Answer

A)  Decreased 
B)  Increased 
C)  Normal 
D)  All of the above 
E)  None of the above

Question 20

The presence of what gene mutation is one of the major citeria for diagnosis of PV?

Accepted Answer

A)  Philadelphia chromosome 
B)  BCR-ABL 
C)  JAC2V617F 
D)  RAS 
E)  MML

Exam 18: Chronic Myeloproliferative Disorders Ii: Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis

Almost all patients with this CMPD are positive for the JAK2(V617F) mutation:

The myeloproliferative disorder typically associated with the highest platelet count [>1 million/L (1000  10⁹/L) ] is:

In the hypercellular (prefibrotic) phase of chronic idiopathic myelofibrosis (CIMF) , the bone marrow displays panhyperplasia with a predominance of __________.

A patient diagnosed with myelofibrosis secondary to gastrointestinal disease may develop a __________ anemia due to excessive blood loss.

What is the hallmark physical finding in CMPDs?

How is essential thrombocythemia differentiated from chronic myelocytic leukemia?

Which clinical manifestations in polycythemia vera are consistent with those of other myeloproliferative syndromes?

Metabolic consequences of myelofibrosis include all of the following except:

When essential thrombocythemia leads to iron deficiency anemia from associated bleeding, a __________ anemia will result.

In polycythemia vera, therapeutic phlebotomies reduce the total red cell mass by removing 350-500 mL over 2-3 days until the hematocrit is maintained at __________ percent.

A __________ anemia is found in most myelofibrosis patients at presentation:

Which is the expected platelet count in essential thrombocythemia?

Hemorrhage and thrombosis are physiologic complications of __________ due to excessive proliferation of the megakaryocyte lineage.

Tissue hypoxia and increased erythropoietic activity is an example of ___________ erythrocytosis.

What is the usefulness of myelosuppression as a treatment in polycythemia vera?

Which of the following are causes of reactive thrombocytosis?

In the absolute erythrocytosis, the red cell mass is __________.

In secondary erythrocytosis, the underlying mechanism is release of __________ as part of a compensatory effect to minimize impending tissue hypoxia.

In both absolute erythrocytosis and relative erythrocytosis the hematocrit is __________.

The presence of what gene mutation is one of the major citeria for diagnosis of PV?

Exam 18: Chronic Myeloproliferative Disorders Ii: Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis

Almost all patients with this CMPD are positive for the JAK2(V617F) mutation:

The myeloproliferative disorder typically associated with the highest platelet count [>1 million/L (1000  109/L) ] is:

In the hypercellular (prefibrotic) phase of chronic idiopathic myelofibrosis (CIMF) , the bone marrow displays panhyperplasia with a predominance of __________.

A patient diagnosed with myelofibrosis secondary to gastrointestinal disease may develop a __________ anemia due to excessive blood loss.

What is the hallmark physical finding in CMPDs?

How is essential thrombocythemia differentiated from chronic myelocytic leukemia?

Which clinical manifestations in polycythemia vera are consistent with those of other myeloproliferative syndromes?

Metabolic consequences of myelofibrosis include all of the following except:

When essential thrombocythemia leads to iron deficiency anemia from associated bleeding, a __________ anemia will result.

In polycythemia vera, therapeutic phlebotomies reduce the total red cell mass by removing 350-500 mL over 2-3 days until the hematocrit is maintained at __________ percent.

A __________ anemia is found in most myelofibrosis patients at presentation:

Which is the expected platelet count in essential thrombocythemia?

Hemorrhage and thrombosis are physiologic complications of __________ due to excessive proliferation of the megakaryocyte lineage.

Tissue hypoxia and increased erythropoietic activity is an example of ___________ erythrocytosis.

What is the usefulness of myelosuppression as a treatment in polycythemia vera?

Which of the following are causes of reactive thrombocytosis?

In the absolute erythrocytosis, the red cell mass is __________.

In secondary erythrocytosis, the underlying mechanism is release of __________ as part of a compensatory effect to minimize impending tissue hypoxia.

In both absolute erythrocytosis and relative erythrocytosis the hematocrit is __________.

The presence of what gene mutation is one of the major citeria for diagnosis of PV?

The myeloproliferative disorder typically associated with the highest platelet count [>1 million/L (1000  10⁹/L) ] is: