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

Question 1

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

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

Answer 1

A and B are correct

Question 2

Which myeloproliferative disorder is characterized by fibrosis of the marrow, extramedullary hematopoiesis of the spleen or liver, leukoerythroblastosis, and teardrop poikilocytosis?

A) Secondary polycythemia
B) Essential thrombocythemia
C) Idiopathic myelofibrosis
D) Polycythemia vera
E) Chronic myelogenous leukemia

Answer 2

Idiopathic myelofibrosis

Question 3

Platelet dysfunction in myelofibrosis may eventually lead to disseminated intravascular coagulation (DIC) suggested by which coagulation abnormalities?

A) Prolonged thrombin time
B) Shortened prothrombin time
C) Elevated fibrin degradation products
D) A and B
E) A and C

Answer 3

A and C

Question 4

According to the 2008 WHO criteria for diagnosis of Polycythemia Vera (PV), the hemoglobin value must be greater than what value for men?

A) 16.5 g/dL
B) 18.5 g/dL
C) 19.5 g/dL
D) 16.0 g/dL
E) 15.5 g/dL

Question 5

Metabolic consequences of myelofibrosis include all of the following except:

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

Question 6

What is/are the strongest differentiating feature(s) that distinguish chronic myelocytic leukemia (CML) from IMF?

A) Decreased leukocyte alkaline phosphatase (LAP)
B) Presence of Philadelphia chromosome
C) Splenomegaly
D) A and B
E) B and C

Question 7

What is meant by myeloid metaplasia in idiopathic myelofibrosis?

A) Myeloid precursors in the bone marrow
B) Intramedullary hematopoiesis in the bone marrow (BM)
C) Intramedullary hematopoiesis in the spleen and liver
D) Extramedullary hematopoiesis in the spleen and liver
E) Erythroid precursors in the BM

Question 8

Bone marrow aspiration in myelofibrosis frequently results in a __________.

A) Clotted specimen
B) Dry tap
C) Hemolysis
D) Normal aspirate
E) None of the above

Question 9

In the fibrotic (f-CIMF) phase of CIMF, which of the following is characteristic?

A) Fibrosis of the marrow
B) Extramedullary hematopoiesis
C) Leukoerythroblastosis of the peripheral blood
D) Splenomegaly
E) All of the above

Question 10

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

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

Question 11

Which of the following characterizes the BM in the prefibrotic stage (p-CIMF) of CIMF according to the World Health Organization (WHO)?

A) Decrease in erythroid precursors
B) Increase in granulocytic and megakaryocytic proliferation
C) Lack of fibrosis
D) Atypical megakaryocytes
E) All of the above

Question 12

Which of the following statements best describes the etiology of the myeloproliferative disorders?

A) An intracorpuscular defect due to an abnormality in hematopoietic cell membrane
B) A neoplastic transformation of a single multipotential stem cell
C) An inherited disorder caused by various enzyme defects in lipid metabolism
D) A lymphocytic malignancy
E) An inherited bone marrow defect

Question 13

Anemia in CIMF is a collective result of:

A) Bone marrow failure
B) Ineffective erythropoiesis
C) Dyserythropoiesis
D) All of the above
E) None of the above

Question 14

All of the following are included in the classic findings of chronic idiopathic myelofibrosis (CIMF) except:

A) Extramedullary hematopoiesis
B) Fibrosis of the marrow
C) Leukoerythroblastosis of the peripheral blood
D) Teardrop poikilocytosis of the peripheral blood
E) Erythroid hyperplasia

Question 15

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

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

Question 16

According to the 2008 WHO criteria for diagnosis of Polycythemia Vera (PV), the hemoglobin value must be greater than what value for women?

A) 16.5 g/dL
B) 18.5 g/dL
C) 19.5 g/dL
D) 14.0 g/dL
E) 15.5 g/dL

Question 17

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

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

Question 18

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

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

Question 19

A patient with myelofibrosis may develop early satisfaction for food intake due to:

A) Thrombocytopenia
B) Hepatomegaly
C) Splenomegaly
D) Anemia
E) Infiltration of fibrotic cells in the stomach

Question 20

In relative erythrocytosis, the red cell mass is __________.

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

Question 21

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

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

Question 22

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

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

Question 23

Which conditions are known to cause a possible false diagnosis of polycythemia vera?

A) Heavy smoking
B) Alcoholic liver disease
C) Systemic lupus erythematosus
D) A and C
E) A and B

Question 24

Which factors constitute a diagnosis of polycythemia vera by the polycythemia vera study group guidelines?

A) Elevated red cell mass
B) Normal arterial oxygen saturation
C) Splenomegaly
D) All of the above
E) None of the above

Question 25

Relative erythrocytosis can be differentiated from secondary erythrocytosis and polycythemia vera by what clinical feature(s)?

A) Lung disease
B) Cyanosis
C) Hepatomegaly
D) Normal red cell mass
E) Normal LAP

Question 26

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

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

Question 27

Dehydration is an example of __________ erythrocytosis.

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

Question 28

Polycythemia vera is characterized by:

A) Suppression of erythropoiesis and excessive proliferation of myeloid elements of the bone marrow
B) Accelerated erythropoiesis and excessive proliferation of myeloid and megakaryocytic elements of the bone marrow
C) Accelerated erythropoiesis and excessive proliferation of lymphoid elements in the bone marrow
D) Suppression of erythropoiesis and excessive proliferation of lymphoid elements of the bone marrow
E) All of the above

Question 29

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

Question 30

What is the objective of polycythemia vera therapy?

A) Reduction of total red cell mass
B) Expansion of total red cell mass
C) Cytotoxic myelosuppression to control malignancy
D) A and B
E) A and C

Question 31

The clinical features of polycythemia vera are attributed to the excessive proliferation of which cell line(s)?

A) Erythrocyte
B) Platelet
C) Granulocyte
D) A and B
E) All of the above

Question 32

Common physical findings in polycythemia vera include:

A) Facial plethora
B) Pruritus
C) Cerebral circulating disturbances
D) Thrombotic episodes
E) All of the above

Question 33

In polycythemia vera, erythropoietin is __________.

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

Question 34

Polycythemia vera has an insidious onset and is most often discovered through a __________ determination.

A) White blood cell (WBC) count
B) Hematocrit
C) Reticulocyte count
D) Platelet count
E) Electrophoresis

Question 35

The presence of splenomegaly in patients with polycythemia vera is due to __________.

A) Intramedullary hematopoiesis
B) Extramedullary hematopoiesis
C) Erythrocytopenia
D) Myeloid metaplasia
E) All of the above

Question 36

A patient presents with a hematocrit of 55%, elevated red cell mass, normal arterial oxygen saturation, elevated LAP score, and thrombocytosis. What is the most probable cause of these laboratory findings?

A) Secondary erythrocytosis
B) Polycythemia vera
C) Relative erythrocytosis
D) Idiopathic myelofibrosis
E) Essential thrombocythemia

Question 37

Rapid reduction of the blood volume to normal can be accomplished by __________.

A) Therapeutic phlebotomy
B) Blood transfusion
C) Plasmapheresis
D) Chemotherapy
E) Bone marrow transplant

Question 38

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

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

Question 39

What is the etiology of polycythemia vera?

A) A physiologic response to an abnormal stimulus
B) Clonal hematologic malignancy of bone marrow
C) Idiopathic cause
D) An inherited hemoglobinopathy
E) An inherited oxygen affinity disorder

Question 40

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

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

Question 41

What bone marrow findings are usually seen in essential thrombocythemia?

A) Trilineage hyperplasia
B) Large megakaryocytes
C) Stainable iron
D) Dysplastic megakaryocyte
E) All of the above

Question 42

Which is the expected platelet count in essential thrombocythemia?

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

Question 43

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

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

Question 44

What is the hallmark physical finding in CMPDs?

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

Question 45

Which of the following are causes of reactive thrombocytosis?

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

Question 46

Essential thrombocythemia is a rare, chronic myeloproliferative disorder characterized by marked __________ associated with abnormal platelet function.

A) Thrombocytopenia
B) Thrombocytosis
C) Erythrocytosis
D) Granulocytosis
E) Lymphocytosis

Question 47

Which laboratory features are helpful in supporting a diagnosis of CIMF instead of essential thrombocythemia?

A) Teardrop poikilocytosis
B) Leukoerythroblastic blood picture
C) Increased prominent fibrosis
D) All of the above
E) B and C

Question 48

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

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

Question 49

Gaisböck's syndrome refers to hypertensive patients who present with increased red cell counts, plethora, and absence of splenomegaly. This relative polycythemia disorder is commonly referred to as:

A) Stress erythrocytosis
B) Malignant erythrocytosis
C) Secondary erythrocytosis
D) Polycythemia vera
E) Primary erythrocytosis

Question 50

The Polycythemia Vera Study Group's (PVSG) updated criteria for the platelet count for diagnosis of essential thrombocythemia (ET) is which of the following?

A) >400,000/µL
B) >700,000/µL
C) >100,000/µL
D) >600,000/µL
E) >500,000/µL

Question 51

Persons living in high altitudes may present with what clinical features due to tissue hypoxia?

A) Increased hematocrit
B) Ruddy cyanosis
C) Normocytic/normochromic erythrocytosis
D) Increased reticulocytes
E) All of the above

Question 52

At onset of disease, essential thrombocythemia will present with a _____________ anemia.

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

Question 53

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

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

Question 54

Which phrase best describes the etiology of essential thrombocythemia?

A) Chromosome defect
B) Physiologic response to an abnormal stimulus
C) Clonal disorder of the multipotential stem cell
D) Amino acid substitution in hemoglobin variants
E) None of the above

Question 55

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

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

Question 56

How is essential thrombocythemia differentiated from chronic myelocytic leukemia?

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