Question 1

The bone marrow fibrosis observed in primary myelofibrosis is due to:

Accepted Answer

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 
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 C

Question 2

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

Accepted Answer

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 
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 B

Question 3

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

Accepted Answer

A)  Increased white count 
B)  Increased red cell mass 
C)  Bone marrow erythroid hyperplasia 
D)  Decreased arterial oxygen saturation 
A)  Increased white count 
B)  Increased red cell mass 
C)  Bone marrow erythroid hyperplasia 
D)  Decreased arterial oxygen saturation D

Question 4

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

Accepted Answer

A)  Secondary polycythemia 
B)  Chronic myelogenous leukemia 
C)  Essential thrombocythemia 
D)  Primary myelofibrosis 
A)  Secondary polycythemia 
B)  Chronic myelogenous leukemia 
C)  Essential thrombocythemia 
D)  Primary myelofibrosis

Question 5

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

Accepted Answer

A)  80% Blasts in the peripheral blood 
B)  Micromegakaryocytes in the peripheral blood 
C)  Autoantibodies 
D)  Extramedullary hematopoiesis 
A)  80% Blasts in the peripheral blood 
B)  Micromegakaryocytes in the peripheral blood 
C)  Autoantibodies 
D)  Extramedullary hematopoiesis

Question 6

Which of the following is true for chronic myelogenous leukemia?

Accepted Answer

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. 
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 7

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

Accepted Answer

A)  Acute myeloid leukemia 
B)  Chronic myelogenous leukemia 
C)  Polycythemia vera 
D)  Essential thrombocythemia 
A)  Acute myeloid leukemia 
B)  Chronic myelogenous leukemia 
C)  Polycythemia vera 
D)  Essential thrombocythemia

Question 8

The Philadelphia chromosome:

Accepted Answer

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 
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 9

Chronic myelogenous leukemia often progresses to:

Accepted Answer

A)  Chronic neutrophilic leukemia 
B)  Chronic lymphocytic leukemia 
C)  Acute leukemia, either myeloid or lymphoblastic 
D)  Chronic idiopathic myelofibrosis 
A)  Chronic neutrophilic leukemia 
B)  Chronic lymphocytic leukemia 
C)  Acute leukemia, either myeloid or lymphoblastic 
D)  Chronic idiopathic myelofibrosis

Question 10

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

Accepted Answer

A)  Essential thrombocytosis 
B)  Polycythemia vera 
C)  Chronic myelogenous leukemia 
D)  All of the above 
A)  Essential thrombocytosis 
B)  Polycythemia vera 
C)  Chronic myelogenous leukemia 
D)  All of the above

Question 11

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

Accepted Answer

A)  Splenomegaly 
B)  Bleeding and thrombosis 
C)  Fatigue 
D)  Infections 
A)  Splenomegaly 
B)  Bleeding and thrombosis 
C)  Fatigue 
D)  Infections

Question 12

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

Accepted Answer

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 
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 13

What do all chronic myeloproliferative disorders share?

Accepted Answer

A)  Philadelphia chromosome 
B)  Increased red cell mass 
C)  Increased blood cells; overlapping clinical and laboratory features 
D)  Serious thromboembolic complications 
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 an expected finding in polycythemia vera?

Accepted Answer

A)  Thrombocytosis 
B)  Anemia 
C)  Low leukocyte alkaline phosphatase 
D)  Normal white count 
A)  Thrombocytosis 
B)  Anemia 
C)  Low leukocyte alkaline phosphatase 
D)  Normal white count

Question 15

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?

Accepted Answer

A)  Polycythemia vera 
B)  Chronic myelogenous leukemia 
C)  Primary myelofibrosis 
D)  Essential thrombocythemia 
A)  Polycythemia vera 
B)  Chronic myelogenous leukemia 
C)  Primary myelofibrosis 
D)  Essential thrombocythemia

Question 16

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

Accepted Answer

A)  Chronic myelogenous leukemia 
B)  Primary myelofibrosis 
C)  Plasma cell myeloma 
D)  Polycythemia vera 
A)  Chronic myelogenous leukemia 
B)  Primary myelofibrosis 
C)  Plasma cell myeloma 
D)  Polycythemia vera

Question 17

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

Accepted Answer

A)  Polycythemia vera 
B)  Bone marrow aplasia 
C)  Dehydration 
D)  Erythrocytosis secondary to hypoxia 
A)  Polycythemia vera 
B)  Bone marrow aplasia 
C)  Dehydration 
D)  Erythrocytosis secondary to hypoxia

Question 18

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

Accepted Answer

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. 
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 19

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

Accepted Answer

A)  Polycythemia vera 
B)  Chronic myelogenous leukemia 
C)  Essential thrombocythemia 
D)  Primary myelofibrosis 
A)  Polycythemia vera 
B)  Chronic myelogenous leukemia 
C)  Essential thrombocythemia 
D)  Primary myelofibrosis

Question 20

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?

Accepted Answer

A)  Essential thrombocythemia 
B)  Chronic myelogenous leukemia 
C)  Thrombocytosis secondary to blood loss 
D)  Polycythemia vera 
A)  Essential thrombocythemia 
B)  Chronic myelogenous leukemia 
C)  Thrombocytosis secondary to blood loss 
D)  Polycythemia vera

Exam 34: Myeloproliferative Neoplasms

The bone marrow fibrosis observed in primary myelofibrosis is due to:

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

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

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

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

Which of the following is true for chronic myelogenous leukemia?

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

The Philadelphia chromosome:

Chronic myelogenous leukemia often progresses to:

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

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

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

What do all chronic myeloproliferative disorders share?

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

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?

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

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

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

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

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?

An Overview of Clinical Laboratory Hematology

Safety in the Hematology Laboratory

Specimen Collection

Care and Use of the Microscope

Quality Assurance in Hematology and Hemostasis Testing

Cellular Structure and Function

Hematopoiesis

Erythrocyte Production and Destruction

Energy Metabolism and Membrane Physiology of the Erythrocyte

Hemoglobin Metabolism

Iron Metabolism

Leukocyte Development, Kinetics, and Functions

Platelet Production, Structure, and Function

Routine and Point-Of-Care Testing in Hematology: Manual and Semiautomated Methods

Examination of the Peripheral Blood Film and Correlation With the Complete Blood Count

Bone Marrow Examination

Body Fluids in the Hematology Laboratory

Anemias: Red Blood Cell Morphology and Approach to Diagnosis

Disorders of Iron and Heme Metabolism

Anemias Caused by Defects of Dna Metabolism

Bone Marrow Failure

Introduction to Increased Destruction of Erythrocytes

Intrinsic Defects Leading to Increased Erythrocyte Destruction

Extrinsic Defects Leading to Increased Erythrocytedestructionnonimmune Causes

Extrinsic Defects Leading to Increased Erythrocytedestructionimmune Causes

Hemoglobinopathies Structural Defects in Hemoglobin

Thalassemias

Nonmalignant Leukocyte Disorders

Introduction to Leukocyte Neoplasms

Cytochemistry

Cytogenetics

Molecular Diagnostics in the Clinical Laboratory

Flow Cytometric Analysis in Hematologic Disorders

Myelodysplastic Syndromes

Acute Leukemias

Mature Lymphoid Neoplasms

Pediatric and Geriatric Hematology

Automated Cell-Counting Instrumentation

Normal Hemostasis and Coagulation

Hemorrhagic Coagulation Disorders

Thrombosis Risk Testing

Thrombocytopenia and Thrombocytosis

Qualitative Disorders of Platelets and Vasculature

Laboratory Evaluation of Hemostasis

Monitoring Antithrombotic Therapies

Coagulation Instrumentation

Filters

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?