Myeloproliferative Neoplasms Flashcards Preview

Hematology Module 2 > Myeloproliferative Neoplasms > Flashcards

Flashcards in Myeloproliferative Neoplasms Deck (41)
Loading flashcards...
0

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

Myeloproliferative neoplasms

1

MPNs are predominantly ________ , not acute

chronic

2

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

CML

3

disease with overproduction of erythrocytes

PV

4

Disease with increased megakaryopoiesis and thrombocytosis

ET

5

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

PMF

6

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

CML Chronic Myelogenous Leukemia

7

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

22

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

BCR/ABL1

9

Laboratory findings in CML PB

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

10

this enzyme is found within secondary granules of neutrophils

Leukocyte ALKALINE phosphatase (LAP)

11

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

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

12

What is a normal LAP score?

Normal 20-100

13

Is the LAP increased or decreased in untreated CML?

decreased

14

Is the LAP increased or decreased in a leukemoid reaction?

normal to increased

15

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

Increased

16

What do lab findings look like in CML Bone Marrow?

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

17

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

chronic; 10%

18

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

Accelerated

19

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

20%

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 ______

myeloid or lymphoid

21

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

NEG; POS

22

How is CML treated?

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

24

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

Imatinib

25

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

CNL has an increased LAP (not decreased like CML)

26

What is Polycythemia Vera's mutation?

JAK2 V617F

27

PV has in increase in what?

RBCs/neutrophils/platelets

28

Main clinical presentation of PV

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

29

PV bone marrow manifestations

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

30

What is the serum EPO level in PV?

below the reference range