Myeloproliferative Neoplasms

Question 0

MPNs are predominantly ________ , not acute

Answer 0

chronic

Question 1

clonal hematopoietic stem cell diseases with expansion, excessive production and over accumulation of erythrocytes, granulocytes and platelets in some combination; usually aquired

Answer 1

Myeloproliferative neoplasms

Question 2

disease with overproduction of granulocytes; the cells do differentiate; clonal disorder of HSC (hematopoietic stem cell line)

Answer 2

CML

Question 3

disease with overproduction of erythrocytes

Answer 3

PV

Question 4

Disease with increased megakaryopoiesis and thrombocytosis

Answer 4

ET

Question 5

combination of overproduction of hematopoietic cells and ineffective hematopoiesis with resultant PB cytopenias; fibrosis in BM

Answer 5

PMF

Question 6

characterized by acquired structural chromosomal abnormality: Philadelphia chromosome t(9;22)(q34;q11.2), creates the fusion gene BCR-ABL1

Answer 6

CML Chronic Myelogenous Leukemia

Question 7

Which one is the derivative chromosome in t(9;22) in CML?

Answer 7

22

Question 8

Unique chimeric gene in CML that codes from a protein with enhanced tyrosine kinase activity which leads to increased proliferation and a loss of apoptotic functions

Answer 8

BCR/ABL1

Question 9

Laboratory findings in CML PB

Answer 9

MK’d leukocytosis, bimodal granulocyte pattern (hallmark), basophilia (Eos can be higher too), Rare nRBC, +/- micromegakaryocytes, increased LD & Uric acid, decreased LAP (stain)

Question 10

this enzyme is found within secondary granules of neutrophils

Answer 10

Leukocyte ALKALINE phosphatase (LAP)

Question 11

how do you score the LAP stain? KNOW HOW TO CALCULATE

Answer 11

score 100 cells from 0-4+ and total the scores; 4+ being a cell that has a lot of secondary granules

Question 12

What is a normal LAP score?

Answer 12

Normal 20-100

Question 13

Is the LAP increased or decreased in untreated CML?

Answer 13

decreased

Question 14

Is the LAP increased or decreased in a leukemoid reaction?

Answer 14

normal to increased

Question 15

Is the LAP increased or decreased in Pregnancy and Polycythemia Vera (PV)?

Answer 15

Increased

Question 16

What do lab findings look like in CML Bone Marrow?

Answer 16

Marked hypercellularity, decreased normoblasts (decreased RBC production), normal to increased megakaryocytes, pseudo-gaucher cells often, and increased reticulin fibrosis

Question 17

Most patients are diagnosed in the ______ phase of CML, where blasts are <_____%

Answer 17

chronic; 10%

Question 18

The ______ phase of CML has a poorer response to therapy, increased anemia, increased clinical symptoms, micromegakaryocytes, etc

Answer 18

Accelerated

Question 19

Blast crisis is classified as >____% Blasts; worst prognosis

Answer 19

20%

Question 20

In CML blast crisis extramedually tissues such as the liver and spleen are involved; since the stem cell is affected, blasts can go either way ______ or ______

Answer 20

myeloid or lymphoid

Question 21

Patients who are Philadelphia Chrom _______ have a worse prognosis than Ph _______ because it progresses more rapidly

Answer 21

NEG; POS

Question 22

How is CML treated?

Answer 22

Synthetic tyrosine kinase inhibitors such as Imatinib (Gleevec), BM transplant, chemo to reduce WBC

Question 24

This drug competes for the binding site of the BCR-ABL gene and reduces activity of the tyrosine kinase

Answer 24

Imatinib

Question 25

Chronic Neutrophilic Leukemia is not CML but its close. What is a big difference between the two?

Answer 25

CNL has an increased LAP (not decreased like CML)

Question 26

What is Polycythemia Vera's mutation?

Answer 26

JAK2 V617F

Question 27

PV has in increase in what?

Answer 27

RBCs/neutrophils/platelets

Question 28

Main clinical presentation of PV

Answer 28

Pruritus (but also CNS probs, vision, heart occlusions, ruddy complexion, ulcers, weight loss, splenomegaly)

Question 29

PV bone marrow manifestations

Answer 29

hypocellular, but M:E is normal BM iron store are decreased Megakaryocytes are increased

Question 30

What is the serum EPO level in PV?

Answer 30

below the reference range

Question 31

Three stages of PV

Answer 31

Prodromal: mild erythrocytosis Overt: significant increased red cell mass Spent: pancytopenia, progresive splenmegaly, BM fibrosis and progression to acute leukemia in 15% of patients

Question 32

Therapy for PV?

Answer 32

Phlebotomy

Question 33

ET female to male ratio?

Answer 33

2:1

Question 34

5 criteria for diagnosing ET?

Answer 34

- platelet count >450 - prolif of megakaryocytic line - meet criteria for MPN,MDS or Myeloid neoplasm - Positive for JAK2 - Presence of BM iron (help dif from PV)

Question 35

Clinical presentation of ET

Answer 35

- erythromyalgia - seizures - MI - dizziness - Platelet dysfunction

Question 36

ET treatment

Answer 36

combination of hydroxyurea and low dose aspirin

Question 37

characterized by proliferation of megakaryocytic and granulocytes. At a later stages there is excessive bone marrow fibrosis

Answer 37

Primary Myelofibrosis (PMF)

Question 38

Three progressive stages of PMF

Answer 38

Prefibrotic Stage: no significant increase in BM fibrosis Early Stage: Fibrotic Stage: significant increase in fibrosis

Question 39

PML lab presentation

Answer 39

Leukoerythroblastosis - tear drops - NRBC - Polychromasia - Some macrocytosis - Micromegakaryocytes

Question 40

PML Bone Marrow

Answer 40

Dry tap due to fibrosis

Question 41

PML sometimes transforms into what?

Answer 41

acute leukemia especially acute myelomonocytic leukemia