Hemat Patho Flashcards
BM exam
- BM aspirate
- BM Bx ( more representative , more painful)
indications of BM Bx
- dry tap
- assess bm cellularity (100 - age)
- assess mets in marrow
- lymphoma staging ( marrow + stage 4)
- MPN
- Storage dis (gaucher)
dry tap
- aplastic anemia <25% HSC cells BM
- myelofibrosis
- hairy cell leukemia (honey comb, fried egg)
oncohemat classfn
MPN MP/MDS MDS AL Lymphomas
Acute leukemia Dx
> 20% BLASTS (2.5-3 times RBC size)
either PS or BM
mc AUER rods
mod amt cytoplasm
cyto granules +
multiple nuclei
Myeloblasts
AML
MPO +
SBB +
AML myeloblasts
NSE +
monoblasts
TRAP +
hairy cell leukemia
scant rim cyto
absent granules
single nucleus
BLOCK + PAS
ALL
lymphoblasts
immunophenotyping by?
flow cytometry
streamlining cells in single linear flow
mc blue laser 488nm
- side scatter
- fwd scatter
- side scatter = granules NEUTROPHILS
- fwd scatter = size BLASTS
Characteristic of BM sample
mekakaryocyte
CD 19
CD 3
CD 34
CD 19 B CELL
CD 3 T CELL
CD 34 BLAST
B cell markers 19 20 10 22, 79a
19 PAN B
20 MATURE B
10 IMMATURE B
22, 79a OTHERS
T cell markers
3
4,8,2,5,7
thymus schooling — cyto shows APC
Pan - 3
myeloid markers
- specific
- myeloid ass
- specific MPO
- myeloid ass CD 13,33,117
NK cell
16
56
monocytes
4
64
stem cells
34
99
TdT (terminal deoxytransferase)
Pan LEUCOCYTE
45
LCA
AL
AML
ALL (B&T)
MPAL (mixed pheno AL)
best marker to diff hematolymphoid ca vs mets
45
MPAL types
BALL + AML mc
TALL + AML
BALL + TALL
BALL + TALL + AML all 3 = triphenotypic RAREST
MPN
CML PV ET PMF CEL (eosinophilic) CNL (neutrophilic)
high TLC
shift to left ( immature myeloid in PS)
ass with BASOPHILIA
normal myeloid maturation
CML
CML vs AL
CML - blasts + PM + M + Band
AL = only 90% BLASTS
TLC > 1 lakh
blasts +
LAP score LOW (0-4)
CML
TLC < 1 lac
absent blasts
HIGH LAP (400)
Leukemoid reacn
normal LAP
80-130
low LAP causes
CML
PNH
HIGH LAP causes
leukamoid reacn
HODGKINS
PV (HIGHEST overall)
CML in blast crisis
stages of CML
- chronic (blasts <10%)
- accelerated (10-19%)
- blastic crisis
10-19% blasts
platelet <1lac or >10lac
basophilia >20%
1/3 Dx accelerated CML
> 20% blasts
presence of extramed disease
CML blast crisis
mc phase where CML detected
chronic phase
Dx hallmark of CML
fusiion trascript BCR-ABL by PCR (higher specificity and sens than conventional karyo/cytogenetics) for Ph Ch
BCR
ABL
22
9
bcr-abl Px
p190
p210
p230
p190 POOR BALL
p210 GOOD :) mc CML, AML
p230
hypercellular marrow + predom myeloid lineage
BM in CML
M:E HIGH
normal M:E = 3-15:1
dis of firsts?
CML
- 1st to fusion transcript
- 1st targetted gene Rx
- 1st recurrent cytogenetic abN detected
criteria for complete hemat remission in CML
- TLC normal
- NO immature cell in PS
- baso <5%
- platelet Normal counts
- absent extramedullary dis
most sig Prognostic marker in CML
MMR
major mol remission = real time PCR/q PCR
high Hb reactive causes of erythrocytosis ruled out low S. EPO clonal mutation + BM hypercellular PANMYELOSIS
PV
reactive causes of erythrocytosis
HIGH EPO smoking high alt copd athelete
PV mutation mc
also in ET and PMF
JAK 2 V 617 F
valine to Phenylalanine
PC > 4.5 lac
reactive causes of thrombocytosis r/o
clonal mutations
ET
reactive causes of thrombocytosis
IDA
infections
ET Mutation
- JAK 2 same
- MPL mutation
fibrosis of marrow NOT A PREREQUISITE
tear drop cell
PMF
stages of PMF
- cellular phase : hyper cellular and dysplastic megakaryocytes
- fibrotic phase
IOC MPN
BM Biopsy
not cytogene/PCR/etc
all are useful in MPN except
flow cytometry
pt with CHLOROMAS next best step
PS
- AML
_ CML
MPN/MDS types
- atypical CML
- CMML
- JMML (HbF high)
high TLC + shift to left bcrabl -ve elderly blasts not raised no basophilia
atypical CML
monocytes raised in PS > 1000
bcr abl -ve
HbF +
CMML or JMML
ps pancytopenia
- hypocellular BM (aplastic anemia <25%)
- hyper cellular BM
mc adults
MDS
hypocellular BM (aplastic anemia <25%)
MDS
PNH
megaloblastic anemia
infections
auoimmune
hypercellular in MDS
PNH
M/C
PNH usually progresses to?
aplastic anemia
lap score when AA progresses to PNH?
decreases
all are preleukemic except
PCH!
- pnh
- mds
- aa
AA can progress to all except
- PRCA
- pnh
- mds
- aml
AA can become PRCA?
NO
MDS types
RCUD RCMD RARS RAEB MDS + isolated 5q del
refractory cytopenias
MDS
do not respond to usual Rx
abN in 1 lineage RBC
- ref anemia
- ref leucopenia
- ref thrombocytopenia
> 10% of 1 lineage is AbN
RCUD
ref cytopenia f unilineage dysplasia
> 1 lineage affected
> 10% of each cell line abN
RCMD
ref cytopenia of multilineage dysplasia
erythroid
stains + PRUSSIAN BLUE (PEARLS stain)
>15% erythroid cells RINGED SIDEROBLASTS
RARS
ref anemia with ringed sideroblasts
PS vs BM
<5% 5-9%
5-19% 10-19%
RAEB 1
RAEB 2
ref anemia with excess blasts
mc cytogenetic abN in MDS
good px
PS - thrombocytosis
MDS with isolated 5q del
mc cytogenetic abN MDS overall
5q del
mc cytogenetic abN MDS adults
5q del
mc cytogenetic abN MDS children
monosomy 17
POOR PROGNOSIS
ALL
B
T
BALL mc 85-90%
TALL 10-15%
BALL good Px
1-10y
t(12,21)
HYPER diploidy
BALL poor Px
high TLC high blasts % LN + HSM <1y or >10y males testis+ CNS + t(9;22) p190 bcr abl infantile leukemia hypodiploidy
infantile leukemia
mc t(4;11)
ALL FAB classfn
L1 same size of blasts POORER PX
L2 varying sizes
L3 BURKITTS
Most sig Px marker in ALL
minimal residual dis
Assess chemoRx response
AML types
M0-7
mc AML
lc AML
mc - M2
lc - M7
M7
acute megakaryocytic
CD 41
61
MPO -ve AML
M0, 6, 7
M6
acute ERYTHROLEUKEMIC
CD 36, 71
glycophorin A +ve
best Px AML
M3
M3
acute promyelocytic
mc t(15;17)
PML RARA
CVS # - NBTE