Deck 21: Myeloproliferative Disorders

Question 1

Which of the following would be the best way to differentiate a myeloproliferative disorder from a myelodysplastic disorder?

A)Bone marrow differential
B)Morphology of cells present
C)Number of cells present
D)Amount of fat present

Answer 1

Morphology of cells present

Question 2

Which myeloproliferative disorder typically produces a dramatic increase in all cell lines?

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

Answer 2

PV

Question 3

A routine CBC is ordered on a patient.Results are as follows: -WBC count = 22.0 x 109/L
-PLT count = 957 x 109/L
-RBC count = 5.00 x 1012/L
-PB smear = moderate platelet clumping and giant platelets noted
These results are characteristic of which disorder?

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

Answer 3

ET

Question 4

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

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

Question 5

CML typically is found in which population?

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

Question 6

CML is characterized by which of the following?

A)A shift to the left in WBCs
B)A massive increase in mature WBCs
C)A peripheral blood or bone marrow blast count >20%
D)A shift to the left in RBCs, platelets and WBCs

Question 7

A patient's CBC revealed the following: marked leukocytosis with an increased number of immature granulocytes.A few promyelocytes and blasts were seen.There were also an increased number of eosinophils and basophils with rare immature forms.The hemoglobin and hematocrit were normal but platelets were slightly decreased.Which is disorder is associated with these findings?

A)Leukemoid reaction
B)CML
C)CIMF
D)Severe infection

Question 8

Which age range is nearest to the peak incidence of CML?

A)0-5 years
B)20-25 years
C)35-45 years
D)55-65 years

Question 9

What feature distinguishes CML from a leukemoid reaction?

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 10

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?

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 11

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

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

Question 12

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?

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

Question 13

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?

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 14

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:

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

Question 15

A known CML patient's blood is received in the lab for a routine CBC.The technologist counts 55% blasts in his smear.What is the most likely reason for this occurrence? The patient is in:

A)The chronic phase of CML.
B)The terminal phase of CML.
C)Blast crisis.
D)The transition phase.

Question 16

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?

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

Question 17

What cell appears to be the primary site for the defect in MPD?

A)HSC
B)CFU-GM
C)CFU-G
D)CFU-GEMM

Question 18

The characteristic poikilocyte often seen in the peripheral blood of patients with CIMF that is indicative of extramedullary hematopoiesis is:

A)Drepanocyte.
B)Schistocyte.
C)Spherocyte.
D)Dacryocyte.

Question 19

Which CBC findings would be suggestive of a MPD?

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

Question 20

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

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

Question 21

A 45-year-old patient visits his physician for a routine examination to address his hypertension.The patient has prominent splenomegaly and rosy cheeks.He is also a smoker.Lab values are: -EPO: elevated;
-WBC count: 18.4 x 109/L4;
-RBC count: 6.84 x 1012/L;
-PLT count: 705 x109/L.
Based on these findings, what would you expect to find in the bone marrow?

A)Myeloid hypoplasia
B)Erythroid hypoplasia
C)Erythroid hyperplasia
D)Panmyelosis

Question 22

Which of the following tests would help differentiate ET from other causes of thrombocytosis?

A)Coagulation screening tests
B)Platelet function tests
C)Liver enzymes
D)D-dimer

Question 23

What is the first step in identifying a variant of CML?

A)Rule out other causes of monoclonal cellular proliferation.
B)Perform a bone marrow aspirate.
C)Take an accurate drug history.
D)Perform cytogenetic tests.

Question 24

Increased sensitivity to which cytokine(s) is thought to be partially responsible for the increased megakaryocyte proliferation of ET?

A)GM-CSF
B)IL-3 and IL-6
C)EPO, SCF and IL-4
D)EPO, MPL, and CSF-G

Question 25

Contrast MDS and MPD utilizing laboratory test result information.

Question 26

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?

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

Question 27

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

A)PDGF
B)EGF
C)TGF-β
D)Fibronectin

Question 28

Explain the significance of the Philadelphia chromosome in ALL.

Question 29

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

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

Question 30

What is the role of PDGF in CIMF?

A)Promotes dysplasia of hematopoietic dividing cells
B)Promotes proliferation of bone marrow fibroblasts
C)Inhibits normal hematopoiesis
D)Inhibits erythropoiesis

Question 31

Discuss the role of EDTA in the LAP score evaluation.

Question 32

The myeloproliferative disorder best described by increased leukocytes, immature granulocytes, and decreased LAP with the Philadelphia chromosome present is which of the following?

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

Question 33

What is the significance of detecting only the G6PD A isoenzyme in a G6PD heterozygote patient with a myeloproliferative disorder?

A)It indicates a higher remission rate.
B)It indicates a poorer prognosis.
C)It indicates a clonal proliferation.
D)It has no significance in MPDs.

Question 34

Explain how a variant of CML is identified utilizing laboratory values.

Question 35

Which of the following test results is most consistent with a diagnosis of CIMF?

A)>30% blasts in the peripheral blood
B)High WBC count
C)Dacryocytes in the peripheral blood
D)High PLT count

Question 36

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?

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 37

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

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

Question 38

Which is found in >90% of cases of PV and promotes EPO receptor activation?

A)JAK2(V617F)
B)Bcl-x1
C)BCR/ABL1
D)RAS

Question 39

What laboratory finding distinguishes leukemoid reaction from CML?

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 40

Which is a characteristic of ET that would help distinguish it from reactive thrombocytosis?

A)Transitory elevated platelet count
B)Splenomegaly
C)Normal platelet function
D)No abnormalities in erythrocytes or leukocytes

Question 41

Contrast primary polycythemia and secondary polycythemia based on pathophysiology.