Myeloid Issues Flashcards
what can MDS progress to
bone marrow failure
also acute myeloid leukemia
what does gains and losses in methylation patterns/genes do
dysregulation of epigenetic programs
DNA demethylases (TET’s) do what
DNA demethylation (low levels of methylation)
normal bone marrow cellularity %
100-age
excessive production of immature forms
acute leukemia
excessive production of mature forms
myeloproliferative neoplasm (chronic leukemia)
excessive production of dysplastic forms
myelodysplastic syndrome
no or very little production of any forms
aplastic anemia
MPN can progress to
myelofibrosis, acute leukemia
CML defined by
BCR-Abl1 fusion gene on philadelphia chromosome
CML presents with
leukocytosis
anemia
splenomegaly
t(9;22)
CML: BCR-ABL1 translocation
major breakpoint = p210
CML common
minor breakpoint = p190
ALL common
gleevec targets
increased tyrosine kinase activity of BCR-ABL1 gene
blasts in chronic phase CML
blasts in CML accelerated phase
10-19%
when does CML transform to acute leukemia (blast crisis)
blasts >20%
JAK2 mutation
associated w/ almost all polycythaemia vera.
but is nonspecific
50-60% of ET/PMF
what causes hemorrhages in P. vera
blood vessel dissension and dysfunctional platelets
pathogenesis of primary myelofibrosis
fibrogenetic factors stimulate marrow fibroblast proliferation
% of PMF that transforms to acute leukemia
5-30%
poikilocytosis with tear-drop (dacrocytes)
PMF
osteosclerosis
late-stage PMF
ET risks
increased risk for thrombotic and hemorrhagic events
ET labs
platelet >450 for 6 months
Myelodysplastic syndromes has increased risk of development of
acute myeloid leukemia
etiology (therapy related)
exposure to chemotherapeutic agents (alkylating-type) and/or radiation therapy
lower risk MDS
RCUD, RARS
intermediate risk MDS
RCMD w/ or w/o RS, REAB
high risk MDS
RAEB 2
good risk categories for MDS
normal cytogenetics isolated del(5q), (20q), and Y
poor risk categories for MDS
complex abnormalities, abnormalities on chromosome 7
MDS- nuclear hypolobation
pseudo Pelger-Huet
