CML PT1 Flashcards

1
Q

Clonal hematopoietic disorders caused by genetic mutations that result in expansion, excessive production, and accumulation of mature rbc, plt, and granulocytes

A

Myeloproliferative neoplasms

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2
Q

DNA mutation acquires causes DNA alterations

A

Full t ancestral stem cell

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3
Q

mpn affects cells in

A

common myeloid progenitor

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4
Q

responds strongly to growth signals

A

hypersensitivity

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5
Q

growth factor of proliferation and differention of granulocytes and neutrophils

A

gcsf

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6
Q

leukemia na di common ang accumulation of cells sa tissues

A

Aml

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7
Q

leukemia na common ang accumulation sa spleen, liver, kidneys, lymph nodes, etc.

A

Cml

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8
Q

notable characteristic mutations of mpn

A

cml - t(9;22)
pv - jak2v617f

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9
Q

what is the affected cell line in cml

A

granulocytes

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10
Q

what is the only bcr::abl1-positive mpns

A

chronic myeloid leukemia

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11
Q

what are the bcr::abl1-negative mpns

A

essential thrombocytopenia
polycythemia vera
primary myelofibrosis

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12
Q

cml is diagnose during what phase

A

chronic phase

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13
Q

if cml is left untreated, what will happen

A

it will terminate acute leukemia (aml/all)

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14
Q

this parameter in cml is apparent at scanning microscopy

A

leukocytosis

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15
Q

sensitive and specific screening tool

A

basophilia

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16
Q

what cells are notable in the bone marrow during the chronic phase

A

pseudo-gaucher cells
sea histiocytes

17
Q

during the chronic phase it is hypercellular, with the expansion of what pool

A

granulocytic pool

18
Q

what morphologic changes in cml is decreased

A

erythropoiesis

19
Q

morphologic changes that is common in myeloproliferative involvements than in aml

A

extramedullary tissue

20
Q
  • enzyme found in the secondary granules of neutrophils
  • diff malignant from non-malignant
21
Q

Cml lap score

22
Q

Leukemoid reaction lap score

23
Q

Normal lap score

24
Q

Why is lap helpful in the differentiation of cml and leukemoid reaction

A

In terms of WBC count they are the same (increased). Leukemoid is a leukemia like reaction

25
Cml pathophysiology
malignancy whereas leukemoid reaction is a physiologic response to severe infection
26
reciprocal translocation between the long arms of chromosome 9 and 22
Philadelphia chromosome
27
Codes for enhanced tyrosine kinase activity
bcr::abl1 gene
28
principle of signal transduction
relays cells a message, and that message instructs the cell on what to do (grow, divide, stop dividing)
29
- normal bcr codes for,, and what activity - regulation of cell growth
p160 serine and threonine kinase activity
30
normal abl1 codes for,, and what activity
p125 normal tyrosine kinase activity
31
transfer phosphate from ATP going to the tyrosine
normal tyrosine kinase activity
32
addition of phosphate and activates series of signals that will instruct the cell to proliferate, grow, differentiate, adhere, survive, or stop
phosphorylation
33
- formation of bcr::abl1 gene - codes for enhanced tyrosine kinase activity
Philadelphia chromosome
34
- tells the cell to grow and divide even when there is no need - always turned-on - HYPERACTIVE ENZYME - hypersensitive; activate signal transduction
tyrosine kinase
35
most preferred treatment in cml
bone marrow and stem cell transplantation
36
first line of theraphy for those who does not qualify transplantation
synthetic tyrosine kinase inhibitor (imatinib mesylate)
37
- targets bcr::abl1 protein - blocks the tyrosine kinase activity - reducing signal transduction activation
synthetic tyrosine kinase inhibitor