CML PT2

Question 1

• clonal expansion resulting to an increase in one or more myeloid cells
• lack the philadelphia chromosome

Answer 1

bcr;;abl1 negative

Question 2

erythrocytosis but also shows leukocytosis and thrombocytosis

Answer 2

polycythemia vera

Question 2

thrombocytosis but can also exhibit leukocytosis with a left shift

Answer 2

essential thrombocytopenia

Question 2

t/f: bcr;;abl1 gene affects a single lineage

Answer 2

false - there might be a tri-lineage proliferation

Question 2

• initially leukocytosis with a left shift but can also exhibit thrombocytosis
• slowly develops fibrosis that inhibits hematopoiesis —> pancytopenia

Answer 2

primary myelofibrosis

Question 2

this mutation is necessary in stimulating proliferation and quesence

Answer 2

mpl

Question 2

common mutations in bcr;;abl1 gene

Answer 2

mpl
jakk2v617f
calr

Question 3

common feature among the 3 conditions

Answer 3

thrombocytosis

Question 3

very common mpn

Answer 3

polycythemia vera

Question 3

• mild erythrocytosis
• often asymptomatic and detected incidentally

Answer 3

prodromal phase

Question 3

• proliferative phase
• hyperviscosity symptoms, thrombotic and hemorrhagic episodes
• splenomegaly, pruritus, erythromelalgia

Answer 3

overt polycythemic phase

Question 3

• progressive splenomegaly or hypersplenism and pancytopenia
• post polycythemic myeloid metaplasia –> bone marrow fibrosis, anemia with tear drop cells

Answer 3

spent phase

Question 3

increase in the actual number of rbc, wbc, and plt resulting from increase in production

Answer 3

true pancytosis

Question 4

what mutation is notable in pcv

Answer 4

jak2v617f

Question 4

pcv
- initial manifestation of pv
- independent of normal regulatory mechanisms associated with increased red cell mass

Answer 4

proliferative phase

Question 4

only happens in post polycythemic phase

Answer 4

increase reticulin production

Question 4

bone marrow with increased production in all blood cells

Answer 4

panmyelosis

Question 5

in jak2 they control the kinase activity

Answer 5

JH1 and JH2

Question 6

has the kinase activity

Answer 6

jh1

Question 7

does not have kinase activity

Answer 7

jh2

Question 8

what jh happens the dimerization of epo

Answer 8

jh2 and jh3

Question 8

what will happen if jak2 is activated

Answer 8

it induces phosphorylation of stat proteins

Question 8

• facilitated by jak2 –> stat protein
• causes a gene signal for gene expression to promote proliferation, differentiation, maturation, and inhibition of apoptosis

Answer 8

phosphorylation

Question 8

valine is released in what domain and converts enzyme to its active form

Answer 8

jh2 domain

Question 8

- confirmational change - ducking point, point of dimerization

Answer 8

jak2

Question 8

rare in pcv

Answer 8

jak2 exon12

Question 8

jak2 exon12 is found in what mpn

Answer 8

secondary myelofibrosis

Question 8

high epo levels, no jak2 mutation

Answer 8

secondary polycythemia

Question 9

- normal rbc mass, predominant thrombocytosis - jak2 mutation present, but lacks significant erythrocytosis

Answer 9

essential thrombocytopenia

Question 9

- bcr-abl fusion gene - leukocytosis with left shift, basophilia, splenomegaly

Answer 9

chronic myeloid leukemia

Question 9

- prominent bone marrow fibrosis - leukoerythroblastic reaction in pb

Answer 9

primary myelofibrosis

Question 9

goal of theraphy for pcv

Answer 9

reduce hyperviscosity

Question 10

treatment of choice for low risk patients in pcv

Answer 10

therapeutic phlebotomy

Question 10

treatment for high risk px (young px or women in childbearing age)

Answer 10

pegylated ifn-a

Question 11

- jak2 inhibitor - first line of choice following hydroxyurea intolerance of failure

Answer 11

ruxolitinib

Question 12

- epigenetic modifier - induces apoptosis in neoplastic cells

Answer 12

givinostat

Question 12

clonal mpn: - increased megakaryopoiesis - sustained thrombocytosis

Answer 12

essential thrombocythemia

Question 12

drivers of acute anemia

Answer 12

alkylating agents

Question 13

- if the clot remains attached to the blood vessel - common due to thrombocytosis - problem in sustained increase plt count

Answer 13

vascular occlusion or hemorrhage

Question 14

- common in fingers and toes - triggered when exposed to cold temp. or stress - due to spasms in toes or fingers

Answer 14

reynaud syndrome and gout

Question 15

common cause of death in essential thrombocythemia

Answer 15

bleeding and thrombosis

Question 15

clonal mpn: - increased abnormal megakeryocytes and granulocyte - ineffective hematopoiesis with leukoerythroblastosis

Answer 15

primary myelofibrosis

Question 16

serves as a matrix for structural framework in BM

Answer 16

collagen

Question 16

the process by which the BM is replaced with connective tissue causing fibrosis

Answer 16

myelofibrosis

Question 16

responsible for the production of collagen

Answer 16

fibroblast

Question 17

what stain is used in reticulin fibers

Answer 17

gomori stain

Question 18

cd marker for hsc and helps strongly in myelofibrosis

Answer 18

cd34+

Question 19

- takes a sample of the bm - examine the bm architecture, fibrosis, cellularity, etc.

Answer 19

bone marrow biopsy

Question 19

- takes a sample of bone marrow fluid - used to analyze individual cells

Answer 19

bone marrow aspiration

Question 19

t/f: first line of theraphy in pm works

Answer 19

false

Question 20

most common cause of death in PM

Answer 20

leukemic transformation

Question 21

most common serious infectious agents in CNL

Answer 21

mycobacteria and fungi

Question 22

only curative treatment inCNL

Answer 22

allogenic stem cell transplantation