2S [LEC]: Myeloproliferative Neoplasms

Question 1

Characterized by excessive proliferation of one or more mature myeloid cell lines

Answer 1

Myeloproliferative neoplasms

Question 2

Genetic mutation most commonly associated with chronic myelogenous leukemia

Answer 2

BCR-ABL

Question 3

Mutation implicated in polycythemia vera, primary myelofibrosis, and essential thrombocytopenia

Answer 3

Janus Kinase 2/ JAK2V617F (Jak2)

Question 4

BCR-ABL is also referred to as ___

Answer 4

Philadelphia chromosome

Question 5

This mutation causes the substitution of phenylalanine for valine at position 617 of the gene

Answer 5

JAK2V617F

Question 6

Gene mutation present in BCR-ABL

Answer 6

t(9;22)

Question 7

BCR gene is located on what chromosome?

Answer 7

Chromosome 22

Question 8

ABL gene is located on what chromosome?

Answer 8

Chromosome 9

Question 9

The resulting fusion protein causes increased ___ activity, which promotes cell proliferation

Answer 9

Tyrosine kinase

Question 10

A therapeutic agent that targets the molecular defect by blocking tyrosine kinase activity

Answer 10

Imatinib mesylate

Question 11

T/F: Patients with CML have better prognosis if they do not have the Philadelphia chromosome

Answer 11

False

Question 12

M:E ratio of patients with CML

Answer 12

25:1

Question 13

This CML phase is characterized as indolent

Answer 13

Initial (chronic) phase

Question 14

This CML phase is when patients become refractive to traditional therapy and may precede acute phase

Answer 14

Accelerated phase

Question 15

If the patient has greater than 50 x 10^9/L or reaching 500 x 10^9/ L WBC count with increased mature granulocytic forms, eosinophilia, basophilia, and thrombocytosis, it is possible that the patient has ___

Answer 15

CML

Question 16

The LAP score of CML patients is usually ___

Answer 16

Decreased

Question 17

CML can mimic this condition as they both display a “left” shift

Answer 17

Neutrophilic Leukemoid Reaction

Question 18

The first synthetic tyrosine kinase inhibitor that selectively binds the ATP binding site and thus inhibit the tyrosine kinase activity of the BCR-ABL fusion protein

Answer 18

Imatinib mesylate

Question 19

A condition related to CML where neutrophil granulocytes are present and 10% are immature, which may manifest with hepatosplenomegaly, bleeding, gout, and pruritus

Answer 19

Chronic Neutrophilic Leukemia (CNL)

Question 20

Population predominantly affected by CML

Answer 20

46-53 y/o

Question 21

Population predominantly affected by CNL

Answer 21

67 y/o

Question 22

T/F: CNL affects women more than men

Answer 22

False (CNL affects both sexes equally)

Question 23

Leukemia associated with expansion of monocytes

Answer 23

Chronic monocytic leukemia

Question 24

Leukemia that affects children younger than 4 years of age

Answer 24

Juvenile Myelomonocytic Leukemia

Question 25

Leukemia that presents >1.5 x 10^9/L eosinophil

Answer 25

Chronic eosinophilic leukemia not otherwise specific (CEL-NOS)

Question 26

Proliferation of mast cells, mutation in the KIT gene

Answer 26

Mastocytosis

Question 27

Malignant hyperplasia of the multipotent myeloid stem cell that causes an increase in all cell lines

Answer 27

Polycythemia vera

Question 28

The most constant and striking feature of polycythemia vera is ___

Answer 28

Erythroid hyperplasia

Question 29

Population predominantly affected by polycythemia vera

Answer 29

40-60 y/o, Men

Question 30

Which of the following manifests in polycythemia vera? i. RBC count of 7-10 x 10^12/L ii. hematocrit of <60% iii. increased hgb of >20 g/dL

Answer 30

i & iii (hct in PV is >60%)

Question 31

Which of the following manifests in polycythemia vera? i. high M:E ratio ii. decreased LAP score iii. hypocellular bone marrow iv. increased whole blood viscosity

Answer 31

iv only (PV: normal or low M:E ratio increased LAP score hypercellular bone marrow)

Question 32

A condition with increased RBC mass as an appropriate response to increased EPO due to tissue hypoxia

Answer 32

Secondary polycythemia

Question 33

EPO level in polycythemia vera is ___

Answer 33

Decreased

Question 34

A condition with decreased plasma volume with a normal RBC mass caused by dehydration or shock

Answer 34

Relative (pseudo) polycythemia

Question 35

A 35-year old patient who was found to have renal cell carcinoma, living in Baguio City, and is a long-time smoker presents increased EPO but normal plasma volume, leukocyte count, and platelet count. What could be the possible condition of the patient?

Answer 35

Secondary polycythemia

Question 36

Primary control of polycythemia vera

Answer 36

Therapeutic phlebotomy

Question 37

Removal of units of whole blood or by large-volume erythrocytapheresis

Answer 37

Therapeutic phlebotomy

Question 38

Use of alkylating myelosuppressive agents to reduce the risk of thrombosis and bleeding

Answer 38

Myelosuppressive therapy

Question 39

Treatment with JAK inhibitors

Answer 39

Targeted molecular therapy (momelotinib)

Question 40

Condition with systemic bone marrow fibrosis and extramedullary hematopoiesis

Answer 40

Primary Myelofibrosis with Myeloid Metaplasia

Question 41

A condition in which the marrow is gradually replaced by fibrotic tissue

Answer 41

Myelofibrosis

Question 42

Collagen type predominantly present in the bone marrow of patients with myelofibrosis

Answer 42

Collagen I, III, and IV

Question 43

Reactive process that causes overproduction of collagen that eventually disrupts the normal architecture of the BM and replaces hematopoietic tissue

Answer 43

Myelofibrosis

Question 44

Expected RBC abnormality seen on PBS of patients with myelofibrosis

Answer 44

Nucleated RBCs Teardrop-shaped RBCs

Question 45

If the patient has MMM, the bone marrow aspirate manifests ___, because of reticulin and collagen fibrosis being locked in the marrow content

Answer 45

Dry tap

Question 46

A 51-year old (M) patient presents progressive anemia and enlarged spleen and liver. His CBC revealed decreased platelet count, while his PBS showed dacrocytes. What may be the possible condition of the patient?

Answer 46

Primary Myelofibrosis with Myeloid Metaplasia

Question 47

The least common among myeloproliferative neoplasms

Answer 47

Essential thrombocytosis/ thrombocythemia

Question 48

This condition is characterized by a significant increase in circulating platelets, usually in excess of 600 x 10^9/ L with giant forms, and platelet function abnormalities

Answer 48

Essential thrombocytosis/ thrombocythemia

Question 49

Second peak of cases of Essential thrombocytosis/ thrombocythemia occur in women in which stage of their lives?

Answer 49

Childbearing age

Question 50

Identify the condition: Erythrocytes: Decreased Leukocytes: Extremely increase Platelets: Moderately increased LAP: Decreased *Positive for Ph' chromosome

Answer 50

CML

Question 51

Identify the condition: Erythrocytes: Decreased Leukocytes: Variable Platelets: Variable LAP: Variable Marrow fibrosis: Very increased *Presence of teardrop-shaped RBCs *Increased number of NRBCs

Answer 51

Primary myelofibrosis

Question 52

Identify the condition: Erythrocytes: Extremely increased Leukocytes: Increased Platelets: Moderately increased LAP: Extremely increased Marrow fibrosis: None

Answer 52

Polycythemia vera

Question 53

Identify the condition: Erythrocytes: Normal Leukocytes: Normal Platelets: Extremely increased LAP: Normal/ increased Marrow fibrosis: None

Answer 53

Essential thrombocytosis

Question 54

A 48-year old patient was seen with Gaucher-like macrophages. He also present enlarged spleen and liver, with normal RBC count, platelet count, and lymphocytes. Other peripheral blood parameters such as WBC count, neutrophils, basophils, and eosinophils, are increased. What could be the possible condition of the patient?

Answer 54

Chronic myeloid leukemia

Question 55

Treatment used to quickly reduce the platelet count

Answer 55

Plateletpheresis

Question 56

A treatment preferred with concomitant thrombocytosis and leukocytosis

Answer 56

Hydroxypurea