CML PT1 Flashcards
Clonal hematopoietic disorders caused by genetic mutations that result in expansion, excessive production, and accumulation of mature rbc, plt, and granulocytes
Myeloproliferative neoplasms
DNA mutation acquires causes DNA alterations
Full t ancestral stem cell
mpn affects cells in
common myeloid progenitor
responds strongly to growth signals
hypersensitivity
growth factor of proliferation and differention of granulocytes and neutrophils
gcsf
leukemia na di common ang accumulation of cells sa tissues
Aml
leukemia na common ang accumulation sa spleen, liver, kidneys, lymph nodes, etc.
Cml
notable characteristic mutations of mpn
cml - t(9;22)
pv - jak2v617f
what is the affected cell line in cml
granulocytes
what is the only bcr::abl1-positive mpns
chronic myeloid leukemia
what are the bcr::abl1-negative mpns
essential thrombocytopenia
polycythemia vera
primary myelofibrosis
cml is diagnose during what phase
chronic phase
if cml is left untreated, what will happen
it will terminate acute leukemia (aml/all)
this parameter in cml is apparent at scanning microscopy
leukocytosis
sensitive and specific screening tool
basophilia
what cells are notable in the bone marrow during the chronic phase
pseudo-gaucher cells
sea histiocytes
during the chronic phase it is hypercellular, with the expansion of what pool
granulocytic pool
what morphologic changes in cml is decreased
erythropoiesis
morphologic changes that is common in myeloproliferative involvements than in aml
extramedullary tissue
- enzyme found in the secondary granules of neutrophils
- diff malignant from non-malignant
Lap
Cml lap score
Decreased
Leukemoid reaction lap score
Increase
Normal lap score
15-170
Why is lap helpful in the differentiation of cml and leukemoid reaction
In terms of WBC count they are the same (increased). Leukemoid is a leukemia like reaction
Cml pathophysiology
malignancy whereas leukemoid reaction is a physiologic response to severe infection
reciprocal translocation between the long arms of chromosome 9 and 22
Philadelphia chromosome
Codes for enhanced tyrosine kinase activity
bcr::abl1 gene
principle of signal transduction
relays cells a message, and that message instructs the cell on what to do (grow, divide, stop dividing)
- normal bcr codes for,, and what activity
- regulation of cell growth
p160
serine and threonine kinase activity
normal abl1 codes for,, and what activity
p125
normal tyrosine kinase activity
transfer phosphate from ATP going to the tyrosine
normal tyrosine kinase activity
addition of phosphate and activates series of signals that will instruct the cell to proliferate, grow, differentiate, adhere, survive, or stop
phosphorylation
- formation of bcr::abl1 gene
- codes for enhanced tyrosine kinase activity
Philadelphia chromosome
- tells the cell to grow and divide even when there is no need
- always turned-on
- HYPERACTIVE ENZYME
- hypersensitive; activate signal transduction
tyrosine kinase
most preferred treatment in cml
bone marrow and stem cell transplantation
first line of theraphy for those who does not qualify transplantation
synthetic tyrosine kinase inhibitor (imatinib mesylate)
- targets bcr::abl1 protein
- blocks the tyrosine kinase activity
- reducing signal transduction activation
synthetic tyrosine kinase inhibitor
