Myeloid Neoplasms: Myeloproliferative & AML Flashcards Preview

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Flashcards in Myeloid Neoplasms: Myeloproliferative & AML Deck (21)
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1

20% of Leukemias are chronic myeloid leukemia and presents w/ marked _____cytosis (2x), massive splenomegaly from extramedullary hematopoiesis, and _______philia

Granulocytosis and Leukocytosis

Basophilia

2

Chronic Myeloid Leukemia translocation

BCR:Abl fusion on philadelphia chromosome t(9;22) (also seen in acute lymphoblastic leukemia w/ intermediated prognosis

Also (q34;q11)

3

t(9;22) BCR: Abl creates what fusion protein

P210

4

Phases of CML

Chronic Phase (2-8 years)
Accelerated Phase (10-19% blasts)
Blast Crisis (70% Myeloid AML, 30% ALL)

5

What does Imatinib treat to downregulate the tyrosine kinase activity in Chronic Myeloid Leukemia

Abl binding domain

6

The JAK2 mutation in polycythemia vera causes what to be phosphorylated?

STAT5

7

Treatment of polycythemia vera can include a tyrosine kinase inhibitor called

Ruxolitinib

8

Phases of Polycythemia vera

Proliferatie Phase
Spent Phase
Post Polycythemia Myelofibrosis
AML

9

Primary myelofibrosis mutations

JAK2, Calreticulin

10

Survival of primary myelofibrosis?

3-5 years

11

Characteristic peripheral blood smear of Primary Myelofibrosis

Tear drop shaped RBCs from passing through fibrotic sinusoids (anisopoikilocytosis)

12

5-20% of those w/ Primary myelofibrosis progress to

AML

13

Essential thrombocytosis demonstrates proliferation of

megakaryoctyes

14

Essential thrombocytosis is a proliferation of megakaryocytes in the bone marrow and so will show in increase of what lab values

platelet numbers, platelets will be abnormal

15

Sheets of megakaryocytes seen in essential thrombocytosis, this poses a risk for

thrombosis

16

20% or more blasts is the definition of

Acute Leukemia

17

The presence of Auer Rods seen in

Acute myeloid Leukemia

Auer rods: abnormal crystallized azurophilic granules, particularly in promyelocytic leukemia

18

Terminal deoxynucleotidyl transferase is positive or negative in Acute Myeloid Leukemias?

negative because TdT is used for gene rearrangement in lymphoid precursor cells and isn't as present in myeloid lineage

19

CD markers for AML?

CD13,33,34,117; Sudan Black +, Myeloperoxidase +

TdT negative

20

Genetic Aberrations in AML w/ favorable prognosis

t(15;17) Acute Promyleocytic Leukemia (RAR-a defect)

t(8;21) Favorable prognosis

inv(16) or t(16;16) Automyelomonocytic Leukemia w/abnml eosinophils favorable prognosis

21

Genetic Aberrations in AML w/ poor prognosis

5q-, 7q-

Also: greater than 60yo, leukocytosis >100,000