Deck 24: Myeloproliferative Neoplasms

Question 1

Which of the following is described by the presence of circulating mast cells in the peripheral blood (≥ 10%), mast cells composing > 20% of the nucleated cells in bone marrow, and multiorgan failure:

A) MPN, U
B) Mast cell leukemia
C) PV
D) Clonal hypereosinophilia

Answer 1

Mast cell leukemia

Question 2

Which CBC findings would suggest an MPN?

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

Answer 2

Leukoerythroblastosis, thrombocytosis

Question 3

Interpret the following results: WBC count: 102 × 10⁹/L
Differential:
13% segmented neutrophils
28% band neutrophils
15% metamyelocytes
8% myelocytes
6% promyelocytes
15% lymphocytes
8% basophils
7% monocytes
LAP score: 15 (reference interval: 0-400)
What disease is associated with this WBC differential and LAP score?

A) None
B) CML
C) CNL
D) Leukemoid reaction

Answer 3

CML

Question 4

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 5

A patient's CBC revealed 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 disorder is associated with these findings?

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

Question 6

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 platelet 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 7

CML is typically diagnosed in which patient population?

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

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?

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

Question 9

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

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

Question 10

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 11

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

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

Question 12

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

A) PMF
B) ET
C) MPN, U
D) PV

Question 13

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) PMF
B) CML
C) PV
D) ET

Question 14

The Philadelphia chromosome results from the 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 15

A 45-year-old male patient presents to his physician with a palpable spleen. A CBC reveals an absolute eosinophil count of 2.5 × 10⁹/L. After six weeks, the patient's CBC indicates an absolute eosinophil count of 2.9 × 10⁹/L. Which of the following is a likely diagnosis for this patient?

A) Severe infection
B) Leukemoid reaction
C) MPN, U
D) Clonal hypereosinophilia

Question 16

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

A) HSC
B) GMP
C) Myeloblast
D) CFU-MK

Question 17

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 18

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 19

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

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

Question 20

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

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

Question 21

The presence of which of the following in the bone marrow would lead a clinician to the diagnosis of a myeloproliferative disorder rather than 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 22

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

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

Question 23

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

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

Question 24

A physician strongly suspects CML in a patient and 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/ABL1
D) PAS stain

Question 25

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 26

How would you confirm a variant of CML?

A) Verify BCL/ABL fusion using FISH analysis.
B) Perform a bone marrow aspirate.
C) Take an accurate drug history.
D) Perform cytochemical staining for MPO and PAS.

Question 27

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 28

Which of the following are criteria for systemic mastocytosis?

A) Atypical morphology in >25% of mast cells
B) >25% of all mast cells in the bone marrow immature or atypical
C) Presence of KIT point mutation in mast cells
D) All of the above

Question 29

Explain the significance of the Philadelphia chromosome in ALL.

Question 30

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 31

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 32

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 × 10⁹/L4
RBC: 6.84 × 10¹²/L
PLT: 705 × 10⁹/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 33

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

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

Question 34

Which of the following genes is associated with clonal hypereosinophilia?

A) BCR/ABL1
B) JAK(V617F)
C) c-Kit
D) PDGFRA

Question 35

Which of the following assays differentiate CNL from CML?

A) Hematocrit
B) WBC count
C) LAP
D) Bone marrow fibrosis

Question 36

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

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

Question 37

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

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

Question 38

An abundance of blasts and some mature forms with an apparent decrease in the intermediate maturational stages is referred to as:

A) Acute lymphoid leukemia
B) Chronic myelogenous leukemia
C) Chronic lymphocytic leukemia
D) Leukemic hiatus

Question 39

What laboratory finding distinguishes leukemoid reaction from CML?

A) Platelets normal in CML, abnormal in leukemoid reaction
B) LAP low in CML, increased in leukemoid reaction
C) Blasts only in leukemoid reaction
D) Occasional micromegakaryocytes in leukemoid reaction

Question 40

The role of PDGF in PMF is to:

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

Question 41

Contrast primary polycythemia and secondary polycythemia based on pathophysiology.

Question 42

Contrast MDS and MPN utilizing laboratory test result information.

Question 43

Compare and contrast MPN and MDS with regard to proliferation, differentiation, and cell death.