Myelodysplastic Syndrome & Myeloproliferative Neoplasms Flashcards Preview

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Flashcards in Myelodysplastic Syndrome & Myeloproliferative Neoplasms Deck (31)
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1

List the 2 main features that characterize myelodysplastic syndrome (MDS).

1. Ineffective Hematopoesis
2. Increased risk of transformation to acute leukemia

2

List the 2 clinical scenarios of MDS.

1. Primary, idiopathic MDS- Elderly (median age 70), 3-5/100,000 per year

2. Secondary, therapy related MDS- Diagonses 2-8 years post therapy (DNA alkylating or ionizing radiation), complex karyoptype (part/full del. ch 5/7)

3

List 3 different Signs of MDS that can lead to a diagnosis

1. Persistent peripheral cytopenia in 1+ lineages
2. If only one lineage, it is almost always persistent anemia
3. Persistent cytopenia in 2+ lineages in patients of advanced age

4

List 3 different types of tests that could be performed to make a diagnosis of MDS.

1. morphological evidence of dysplasia (Look at a smear, or karyotype)
2. Increased myeloblasts (but less than 20% of blood/marrow)
3. Presence of clonal cytogenetic abnormalities

5

List 4 possible causes of secondary myelodysplasia that might mimic MDS.

1. Vitamin Deficiency (B12, folate)
2. Toxin Exposure (Heavy metals)
3. Exposure to certain drugs
4. Viral infections

6

Pelgeroid Cells (Image)

Dysplastic neutrophils with two lobes

7

Low Grade MDS

Myeloblasts are not increased in frequency (<2% in blood)

8

High Grade MDS

Myeloblasts are increased in frequency, but less than 20% (myeloblasts account for 5-19% of marrow cells, and/or 3-19% of blood cells)

9

Refractory Cytopenia with Unilineage Dysplasia, RCUD

Low grade
Relatively good prognosis

10

Refractory anemia with Excess Blasts 1- RAEB-1

High Grade

Relatively Dismal prognosis
5-9 in marrow, 2-4 in blood

11

Myeloproliferative Neoplasms (MPNs)

Arise from transformed hematopeotic cells (Like MDS)

Still effective hematopeosis however

Younger patients than MDS

Marrow growing too much

12

List 2 reasons for the frequent occurrence of splenomegaly and hepatomegaly in patients with MPNs.

1. Too many cells, so they're sequestered in the two organs
2. Transition to extramedullary production of blood cells with marrow fibrosis

13

List 3 possible negative end points for MPNs.

1. Excessive marrow fibrosis (marrow aging and scarred)
2. Transform to acute leukemia
3. The two megaly's

14

Refractory Cytopenia with Multilineage Dysplasia (RCMD)

Low Grade
Dysplasia in 2 or more lineages
Worse prognosis than RCUD
10% transform to AML

15

Chronic Myelogenous Leukemia (CML)

Increased WBC (12,000 to 1,000,000)
Neutrophilic leukocytosis

Increased basophils and often platelets

If untreated, goes to blast phase (basically acute leukemia)

16

Polycythemia Vera (PV)

Increased in RBC mass
Sometimes increased neutro's and plts

Transforms to spent phase (fibrotic marrow, bone marrow failure)

17

Primary Myelofibrosis (PMF)

Granulocytic and megakaryocytic hyperplasia

Leukoerythrobastosis- more immature cells than usual (both RBC and WBC)

Teardrops in the bloodsmear because squeezed out marrow

The two megaly's due to extramedullary hematopoesis

18

Essential Thrombocythemia (ET)

Just increased megakaryocytes -> increased plts

19

Refractory Anemia with Excess Blasts-2 (RAEB-2)

High grade
"Very dismal"
10-19% blasts in marrow, and/or 5-19% blasts in blood

20

Explain why there is a need for a second and third generation of protein tyrosine kinase inhibitors (PTKIs).

Cancer has developed resistance through mutations involved imatinib binding sites

21

List 3 sites where thrombosis should always make one consider the possibility of PV.

1.) Mesenteric Vein
2.) Hepatic Portal Vein
3.) Splenic Vein

22

The most common method of death attributable to disease in polycythemia vera (PV) patients

Thrombotic events

23

Describe findings that might be seen in a peripheral blood smear of a patient with leukoerythroblastosis and how these findings relate to patients with marrow fibrosis.

A lot of immature WBCs and RBCs in peripheral blood because the firbrosis is forcing the blasts out of the marrow sooner

Dacryocytes

24

Chronic Myelogenous Leukemia (CML)

Cytogenic Finding

BCR-ABL gene fusion t(9;22), philadelphia chr. fusion protein

25

Chronic Myelogenous Leukemia (CML)

Marrow Finding

Hypercellular bone marrow and granulocytic hyperplasia

26

Polycythemia Vera (PV)

Cytogenic Finding

Point Mutation of JAK2, a cell signalling pathway protein

27

Polycythemia Vera (PV)

Marrow Finding


Marrow is hypercellular, with bizarre megakaryocytes

28

Primary Myelofibrosis (PMF)

Cytogenic Findings

Jak2 mutation in 50%, other 50% have MPL or CALR mutation

29

Primary Myelofibrosis (PMF)

Marrow Findings

Hypercellular marrow
Large, bizarre megakaryocytes

30

Essential Thrombocythemia (ET)

Cytogenic Findings

Jak2 mutation 50% of time, otherwise MPL or CALR