Question 1

What is meant by myeloid metaplasia in idiopathic myelofibrosis?

Accepted Answer

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 2

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

Accepted Answer

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

Question 3

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

Accepted Answer

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

Question 4

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

Accepted Answer

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

Question 5

What is the objective of polycythemia vera therapy?

Accepted Answer

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 6

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

Accepted Answer

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

Question 7

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

Accepted Answer

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 8

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

Accepted Answer

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

Question 9

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

Accepted Answer

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 10

Common physical findings in polycythemia vera include:

Accepted Answer

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

Question 11

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

Accepted Answer

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

Question 12

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?

Accepted Answer

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

Question 13

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

Accepted Answer

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 14

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

Accepted Answer

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

Question 15

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

Accepted Answer

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

Question 16

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

Accepted Answer

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

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