Myelodysplastic Syndrome & Myeloproliferative Neoplasms Flashcards Preview

Lindsey's Blood and Lymph Unit III > Myelodysplastic Syndrome & Myeloproliferative Neoplasms > Flashcards

Flashcards in Myelodysplastic Syndrome & Myeloproliferative Neoplasms Deck (93)
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1

What does the BCR-ABL1 fusion protein do?

constitutive activation of several growth pathways

1

Who gets secondary erythrocytosis?

smokers; people with chronic hypoxia, certain hemoglobin disorders

2

What is plethora?

dusky, reddish skin

3

How is MDS diagnosed?

at least 1 persistent cytopenia; evaluate bone marrow and chromosomes

3

What is CML?

a hematopoietic stem cell disorder assoc. with BCR-ABL1 gene fusion; usually neutrophilic leukocytosis

4

How is CML diagnosed?

presence of BCR-ABL1 via cytogenetics, FISH, or RT-PCR of mRNA

4

Which disease is indolent?

ET

5

Why does hepatomegaly occur in MPN?

extramedullary hematopoeisis

6

Most cases of PV contain the ____ mutation.

V617F point

7

PMF eventually progresses to the ____ stage, in which the bone marrow has reticulin fibrosis, the peripheral blood has leukoerythroblastosis, and other organs enlarge because of extramedullary hematopoiesis.

fibrotic

7

How is ET diagnosed?

incidentally bc of increased platelet counts

8

What is the incidence rate of CML? Avg. age at diagnosis?

1-2/100,000; 40-50yo

9

What is the outcome of CML?

80-85% survival

9

What is ET?

an MPN characterized by sustained thrombocytosis and no granulocytic hyperplasia in the marrow

10

What does PMF stand for?

primary myelofibrosis

10

What are paresthesias?

pins-and-needles sensation

10

In the prefibrotic stage of PMF, the marrow is hypercellular, showing a _____ and _____ proliferation.

granulocytic; megakaryocytic

10

What is the prognosis of PMF? Survival?

poor if diagnosed in fibrotic stage- median survival 5 years

10

What is the prognosis of ET?

good; survival 10-15 years or more; an indolent disease

12

What is RC-UD?

cytopenia of the one of the various lineages (ie anemia, neutropenia)

13

In the ____ stage of PV, peripheral blood cell counts are increased.

polycythemic

15

What does MDS stand for?

myelodysplastic syndrome

15

What are the 2 broad clinical scenarios of MDS?

1. 1a/idiopathic 2. 2a/therapy related (t-MDS)

16

What does PV stand for?

polycythemia vera

17

What karyotypes can be seen in MDS?

monosomy or deletion of 5 or 7; trisomy 8

18

What are MPNs?

proliferations of 1+ myeloid lineages

18

The JAK2 mutation can be found in ____ of cases of PMF.

50%

19

What is the treatment for CML?

PTKIs: imatinib (Gleevac) and dasatinib

20

What would be found in a marrow biopsy of CML in the chronic phase?

hypercellularity; granulocytic hyperplasia; small megakaryocytes with round, non-lobated nuclei; no dysplasia

21

Once MDS has been diagnosed, it is classified as either ____ or ____.

low grade; high grade