WBC Disorders VII Flashcards
3 categories of myeloid neoplasia
1 - acute myeloid leukemia
2 - myelodysplastic syndrome
3 - myeloproliferative disorder
accumulation of immature myeloid forms in bone marrow supress normal hematopoiesis
AML
Dx of AML
20% blasts in bone marrow
ineffective hematopoiesis leads to cytopenia
myelodysplastic syndromes
increased production of one or more types of committed mature white,red cells of myeloid series
myeloproliferative disorders
transform to AML
MDS and MPD
origin from hematopoietic progenitor cell
myeloid neoplasias
CD34
multipotent stem cells
CD64
mature myeloid cells
CD15
mature myeloid cells
AML with prior MDS
poor prognosis
therapy related AML
very poor prognosis
AML, NOS
M0-7 classes
t(8;21) (q22;q22)
favorable prognosis AML
RUNX1/ETO fusion gene
M2
inv(16) (p13;q22)
favorable prognosis AML
CBFB/MKH11 fusion gene
M4eo
t(15;17) (q22;11-12)
intermediate prognosis AML
RARA/PML fusion gene
M3
t(11q23;v)
poor prognosis AML
MLL fusion gene
M4/M5
myeloperoxidase
identify myeloblast
nonspecific
identify monoblasts
auer rods
identify myeloid lineage
myeloperoxidase negative AML
M0 - minimally differentiated
myeloperoxidase positive AML
M1 - AML without maturation
non-specific esterase positive AML
M5 - AML with monocytic maturation
AML path
disruption of gene encoding transcription factors for normal myeloid differentiation
epigenome effects
t(15;17)
acute promyelocytic leukemia
M3
acute myeloid leukemia without maturation
M1 subtype
-flow cytometry
+ for CD34, CD33, CD15 (subset)
- for CD64
myeloid cells showing limited maturation
acute promyelocytic leukemia
M3
t(15;17)
show auer rods
acute myeloid leukemia with monocytic differentiation
M5b subtype
severe bleeding from gums
AML
-acute myeloid leukemia
fatigue, fever, infection, bleeding
clinical for AML
fatigue - anemia
infection - neutropenia
bleeding - thrombocytopenia
granulocytic sarcoma
localized primary tumor mass
with AML
leukemia cutis
monocytic feature
monocytic differentiation - often with skin infiltration
antibodies against GpIIb/IIIa or vWF
AML with megakaryocytic maturation
M7
AML with erythroid maturation
M6
AML with myelomonocytic maturation
M4
AMML
monocytic and myelocytic differentiation
MPO positive
NSE positive
M0
AML minimally differentiated
MPO negative
acute promyelocytic leukemia
M3
- high incidence of DIC
- t(15;17) association
t-MDS
secondary myelodysplasia syndrome
to genotoxic drug or radiation therapy
worse prognosis
and worse cytopenia
mutation at TP53
LOF mutation
in myelodysplasia syndrome
clinical for myelodysplasia
70yo
progress to AML 10-40%
pseudo-peiger-huet cells
neutrophils with only two nuclear lobes
in myelodysplasia
megakaryocytes with multiple nuclei
in myelodysplasia
ringed sideroblasts
erythroid progenitors with iron laden mitochondria seen as blue perinuclear granules
in myelodysplasia
discovered on accident on blood screen
often with myelodysplasia
MDS prognosis
survival 9-29 months
constitutively activated tyrosine kinases
myeloproliferative disorders