Question 1

CML typically is found in which population?

Accepted Answer

A) Male children 
B) Female adults 
C) Male and female elderly patients 
D) Teenagers 
A) Male children 
B) Female adults 
C) Male and female elderly patients 
D) Teenagers

Question 2

Contrast primary polycythemia and secondary polycythemia based on pathophysiology.

Accepted Answer

Primary polycythemia is caused by a muta

Question 3

The presence of which of the following in the bone marrow would lead the clinician to a diagnosis of a myeloproliferative disorder rather than of an acute leukemia?

Accepted Answer

A) High numbers of proliferating mature cells 
B) High numbers of young dividing cells 
C) High amount of bone marrow fat present 
D) High amount of abnormal cells 
A) High numbers of proliferating mature cells 
B) High numbers of young dividing cells 
C) High amount of bone marrow fat present 
D) High amount of abnormal cells

Question 4

The Philadelphia chromosome results from a fusion of which of the following?

Accepted Answer

A) Chromosomes 9 and 22 
B) Chromosomes 15 and 17 
C) Chromosomes 8 and 21 
D) Chromosomes 8 and 14 
A) Chromosomes 9 and 22 
B) Chromosomes 15 and 17 
C) Chromosomes 8 and 21 
D) Chromosomes 8 and 14

Question 5

A patient presents with a moderate leukoerythroblastic anemia, striking anisocytosis and poikilocytosis.The anemia is normocytic, normochromic.Large platelets are noted.Dacryocytes, elliptocytes, and ovalocytes are present.Basophilic stippling is present.What disease is associated with these findings?

Accepted Answer

A) CIMF 
B) CML 
C) PV 
D) ET 
A) CIMF 
B) CML 
C) PV 
D) ET

Question 6

Which of the following tests would help differentiate primary polycythemia from secondary polycythemia?

Accepted Answer

A) EPO 
B) Vitamin B₁₂ 
C) Iron 
D) LAP score 
A) EPO 
B) Vitamin B₁₂ 
C) Iron 
D) LAP score

Question 7

Which CBC findings would be suggestive of a MPD?

Accepted Answer

A) Erythrocytosis, leukopenia, thrombocytopenia 
B) Marked anemia, leukocytosis, thrombocytopenia 
C) Leukoerythroblastosis, thrombocytosis 
D) Leukoerythroblastosis, thrombocytopenia 
A) Erythrocytosis, leukopenia, thrombocytopenia 
B) Marked anemia, leukocytosis, thrombocytopenia 
C) Leukoerythroblastosis, thrombocytosis 
D) Leukoerythroblastosis, thrombocytopenia

Question 8

The most striking finding in the peripheral blood in this disease is extreme and consistent thrombocytosis.Giant, bizarre platelet forms are present in the peripheral blood.Megakaryocytes and megakaryocyte fragments can be present.Abnormalities in platelet aggregation and adhesion are common.What is this disease?

Accepted Answer

A) PV 
B) ET 
C) CML 
D) Mast cell disease 
A) PV 
B) ET 
C) CML 
D) Mast cell disease

Question 9

Discuss the role of EDTA in the LAP score evaluation.

Accepted Answer

EDTA inhibits the activity of

Question 10

What feature distinguishes CML from a leukemoid reaction?

Accepted Answer

A) CML has no blasts in the peripheral blood; in a leukemoid reaction blasts are common. 
B) CML often has abnormal platlet morphology and leukemoid reaction has normal platelets. 
C) LAP is increased in CML and decreased in leukemoid reaction. 
D) Chromosome karyotype is abnormal in leukemoid reaction and normal in CML. 
A) CML has no blasts in the peripheral blood; in a leukemoid reaction blasts are common. 
B) CML often has abnormal platlet morphology and leukemoid reaction has normal platelets. 
C) LAP is increased in CML and decreased in leukemoid reaction. 
D) Chromosome karyotype is abnormal in leukemoid reaction and normal in CML.

Question 11

Explain the significance of the Philadelphia chromosome in ALL.

Accepted Answer

There are two different types of protein

Question 12

A LAP score is ordered on a patient with a WBC count of 68 x 109/L.A fingerstick is performed, and the slides are sent to the lab for processing.The technologist performs the analysis and obtains the following results: Neg (0) = 35
1+ = 25
2+ = 15
3+ = 25
4+ = 0
What is the LAP score? What is the most probable diagnosis based on the LAP score?

Accepted Answer

A) LAP score = 25; diagnosis = CML 
B) LAP score = 5; diagnosis = leukemoid reaction 
C) LAP score = 100; diagnosis = CML 
D) LAP score = 130; diagnosis = leukemoid reaction 
A) LAP score = 25; diagnosis = CML 
B) LAP score = 5; diagnosis = leukemoid reaction 
C) LAP score = 100; diagnosis = CML 
D) LAP score = 130; diagnosis = leukemoid reaction

Question 13

A patient's laboratory results indicate a markedly increased hemoglobin, hematocrit, and red blood cell count.White blood cells and platelets are also slightly increased.The erythropoietin level is decreased.Which is most likely:

Accepted Answer

A) Relative polycythemia. 
B) Secondary polycythemia. 
C) Polycythemia vera. 
D) Reactive erythrocytosis. 
A) Relative polycythemia. 
B) Secondary polycythemia. 
C) Polycythemia vera. 
D) Reactive erythrocytosis.

Question 14

The key factor to distinguish relative and absolute polycythemia is that a relative polycythemia is due to:

Accepted Answer

A) Increased production of erythrocytes. 
B) Decreased amount of serum. 
C) Increased production of erythropoietin. 
D) Decreased arterial oxygen saturation. 
A) Increased production of erythrocytes. 
B) Decreased amount of serum. 
C) Increased production of erythropoietin. 
D) Decreased arterial oxygen saturation.

Question 15

Interpret the following results: -WBC count: 102 x 109/L
-Differential: 13% segmented neutrophils, 28% band neutrophils, 15% metamyelocytes, 8% myelocytes, 6% promyelocytes, 15% lymphocytes, 8% basophils, 7% monocytes
-LAP score: 100 band and segmented neutrophils:
-Cells with a negative (0) reaction = 92
-Cells with a positive 1+ reaction = 5
-Cells with a positive 2+ reaction = 3
What is the LAP score? What disease is associated with this LAP score?

Accepted Answer

A) LAP score = 8; disease = CML 
B) LAP score = 11; disease = CML 
C) LAP score = 103; no disease association 
D) LAP score = 157; disease = leukemoid reaction 
A) LAP score = 8; disease = CML 
B) LAP score = 11; disease = CML 
C) LAP score = 103; no disease association 
D) LAP score = 157; disease = leukemoid reaction

Question 16

Which of the following myeloproliferative disorders is characterized by an elevated white count and a markedly elevated platelet count?

Accepted Answer

A) CML 
B) ET 
C) PV 
D) CIMF 
A) CML 
B) ET 
C) PV 
D) CIMF

Question 17

What laboratory finding distinguishes leukemoid reaction from CML?

Accepted Answer

A) Normal platelets in CML, abnormal in leukemoid reaction 
B) LAP low in CML and increased in leukemoid reaction 
C) Blasts only in leukemoid reaction 
D) Occasional micromegakaryocytes in leukemoid reaction 
A) Normal platelets in CML, abnormal in leukemoid reaction 
B) LAP low in CML and increased in leukemoid reaction 
C) Blasts only in leukemoid reaction 
D) Occasional micromegakaryocytes in leukemoid reaction

Question 18

A physician strongly suspects CML in his patient.He has already ordered a bone marrow differential and cytogenetic analysis for the Philadelphia chromosome.What other test would help confirm the CML picture for this patient?

Accepted Answer

A) Molecular assay for JAK2 kinase 
B) Immunophenotyping with flow cytometry 
C) Molecular assay for BCR-ABL 
D) PAS stain 
A) Molecular assay for JAK2 kinase 
B) Immunophenotyping with flow cytometry 
C) Molecular assay for BCR-ABL 
D) PAS stain

Question 19

What substance(s) is/are primarily responsible for stimulating the growth and proliferation of fibroblasts in CIMF?

Accepted Answer

A) PDGF 
B) EGF 
C) TGF-β 
D) Fibronectin 
A) PDGF 
B) EGF 
C) TGF-β 
D) Fibronectin

Question 20

The major cell line involved in the pathogenesis of CIMF is which of the following?

Accepted Answer

A) Megakaryocytic 
B) Erythrocytic 
C) Myelocytic 
D) Fibroblast 
A) Megakaryocytic 
B) Erythrocytic 
C) Myelocytic 
D) Fibroblast

Exam 21: Myeloproliferative Disorders

CML typically is found in which population?

Contrast primary polycythemia and secondary polycythemia based on pathophysiology.

The presence of which of the following in the bone marrow would lead the clinician to a diagnosis of a myeloproliferative disorder rather than of an acute leukemia?

The Philadelphia chromosome results from a fusion of which of the following?

Which of the following tests would help differentiate primary polycythemia from secondary polycythemia?

Which CBC findings would be suggestive of a MPD?

Discuss the role of EDTA in the LAP score evaluation.

What feature distinguishes CML from a leukemoid reaction?

Explain the significance of the Philadelphia chromosome in ALL.

A patient's laboratory results indicate a markedly increased hemoglobin, hematocrit, and red blood cell count.White blood cells and platelets are also slightly increased.The erythropoietin level is decreased.Which is most likely:

The key factor to distinguish relative and absolute polycythemia is that a relative polycythemia is due to:

Which of the following myeloproliferative disorders is characterized by an elevated white count and a markedly elevated platelet count?

What laboratory finding distinguishes leukemoid reaction from CML?

A physician strongly suspects CML in his patient.He has already ordered a bone marrow differential and cytogenetic analysis for the Philadelphia chromosome.What other test would help confirm the CML picture for this patient?

What substance(s) is/are primarily responsible for stimulating the growth and proliferation of fibroblasts in CIMF?

The major cell line involved in the pathogenesis of CIMF is which of the following?

Introduction

Cellular Homeostasis

Hematopoiesis

Structure and Function of Hematopoietic Organs

The Erythrocyte

Hemoglobin

The Leukocyte

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Hemoglobinopaties: Qualitative Defects

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Introduction to Hemolytic Anemia

Hemolytic Anemia: Membrane Defects

Hemolytic Anemia: Enzyme Deficiencies

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Granulocyte and Monocyte Disorders

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Acute Lymphoblastic Leukemias

Lymphoid Malignancies: Chronic Lymphoid Leukemias, Lymphomas, and Plasma Cell Neoplasms

Hematopoietic Stem Cell Transplantation

Morphologic Analysis of Body Fluids in the Hematology Laboratory

Primary Hemostasis

Secondary Hemostasis and Fibrinolysis

Disorders of Primary Hemostasis

Disorders of Secondary Hemostasis

Thrombophilia

Hematology Procedures and Hemostasis

Bone Marrow Examination

Automation in Hematology and Hemostasis

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Laboratory Testing in Coagulation

Quality Assessment in the Hematology Laboratory

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