Hemat Patho Flashcards

1
Q

BM exam

A
  • BM aspirate

- BM Bx ( more representative , more painful)

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2
Q

indications of BM Bx

A
  • dry tap
  • assess bm cellularity (100 - age)
  • assess mets in marrow
  • lymphoma staging ( marrow + stage 4)
  • MPN
  • Storage dis (gaucher)
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3
Q

dry tap

A
  • aplastic anemia <25% HSC cells BM
  • myelofibrosis
  • hairy cell leukemia (honey comb, fried egg)
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4
Q

oncohemat classfn

A
MPN
MP/MDS
MDS
AL
Lymphomas
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5
Q

Acute leukemia Dx

A

> 20% BLASTS (2.5-3 times RBC size)

either PS or BM

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6
Q

mc AUER rods
mod amt cytoplasm
cyto granules +
multiple nuclei

A

Myeloblasts

AML

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7
Q

MPO +

SBB +

A

AML myeloblasts

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8
Q

NSE +

A

monoblasts

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9
Q

TRAP +

A

hairy cell leukemia

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10
Q

scant rim cyto
absent granules
single nucleus
BLOCK + PAS

A

ALL

lymphoblasts

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11
Q

immunophenotyping by?

A

flow cytometry

streamlining cells in single linear flow

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12
Q

mc blue laser 488nm

  • side scatter
  • fwd scatter
A
  • side scatter = granules NEUTROPHILS

- fwd scatter = size BLASTS

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13
Q

Characteristic of BM sample

A

mekakaryocyte

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14
Q

CD 19
CD 3
CD 34

A

CD 19 B CELL
CD 3 T CELL
CD 34 BLAST

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15
Q
B cell markers 
19
20
10
22, 79a
A

19 PAN B
20 MATURE B
10 IMMATURE B
22, 79a OTHERS

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16
Q

T cell markers
3
4,8,2,5,7

A

thymus schooling — cyto shows APC

Pan - 3

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17
Q

myeloid markers

  • specific
  • myeloid ass
A
  • specific MPO

- myeloid ass CD 13,33,117

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18
Q

NK cell

A

16

56

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19
Q

monocytes

A

4

64

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20
Q

stem cells

A

34
99
TdT (terminal deoxytransferase)

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21
Q

Pan LEUCOCYTE

A

45

LCA

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22
Q

AL

A

AML
ALL (B&T)
MPAL (mixed pheno AL)

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23
Q

best marker to diff hematolymphoid ca vs mets

A

45

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24
Q

MPAL types

A

BALL + AML mc
TALL + AML
BALL + TALL
BALL + TALL + AML all 3 = triphenotypic RAREST

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25
MPN
``` CML PV ET PMF CEL (eosinophilic) CNL (neutrophilic) ```
26
high TLC shift to left ( immature myeloid in PS) ass with BASOPHILIA normal myeloid maturation
CML
27
CML vs AL
CML - blasts + PM + M + Band | AL = only 90% BLASTS
28
TLC > 1 lakh blasts + LAP score LOW (0-4)
CML
29
TLC < 1 lac absent blasts HIGH LAP (400)
Leukemoid reacn
30
normal LAP
80-130
31
low LAP causes
CML | PNH
32
HIGH LAP causes
leukamoid reacn HODGKINS PV (HIGHEST overall) CML in blast crisis
33
stages of CML
- chronic (blasts <10%) - accelerated (10-19%) - blastic crisis
34
10-19% blasts platelet <1lac or >10lac basophilia >20%
1/3 Dx accelerated CML
35
>20% blasts | presence of extramed disease
CML blast crisis
36
mc phase where CML detected
chronic phase
37
Dx hallmark of CML
fusiion trascript BCR-ABL by PCR (higher specificity and sens than conventional karyo/cytogenetics) for Ph Ch
38
BCR | ABL
22 | 9
39
bcr-abl Px p190 p210 p230
p190 POOR BALL p210 GOOD :) mc CML, AML p230
40
hypercellular marrow + predom myeloid lineage
BM in CML M:E HIGH normal M:E = 3-15:1
41
dis of firsts?
CML - 1st to fusion transcript - 1st targetted gene Rx - 1st recurrent cytogenetic abN detected
42
criteria for complete hemat remission in CML
- TLC normal - NO immature cell in PS - baso <5% - platelet Normal counts - absent extramedullary dis
43
most sig Prognostic marker in CML
MMR | major mol remission = real time PCR/q PCR
44
``` high Hb reactive causes of erythrocytosis ruled out low S. EPO clonal mutation + BM hypercellular PANMYELOSIS ```
PV
45
reactive causes of erythrocytosis
``` HIGH EPO smoking high alt copd athelete ```
46
PV mutation mc | also in ET and PMF
JAK 2 V 617 F | valine to Phenylalanine
47
PC > 4.5 lac reactive causes of thrombocytosis r/o clonal mutations
ET
48
reactive causes of thrombocytosis
IDA | infections
49
ET Mutation
- JAK 2 same | - MPL mutation
50
fibrosis of marrow NOT A PREREQUISITE | tear drop cell
PMF
51
stages of PMF
- cellular phase : hyper cellular and dysplastic megakaryocytes - fibrotic phase
52
IOC MPN
BM Biopsy | not cytogene/PCR/etc
53
all are useful in MPN except
flow cytometry
54
pt with CHLOROMAS next best step
PS - AML _ CML
55
MPN/MDS types
- atypical CML - CMML - JMML (HbF high)
56
``` high TLC + shift to left bcrabl -ve elderly blasts not raised no basophilia ```
atypical CML
57
monocytes raised in PS > 1000 bcr abl -ve HbF +
CMML or JMML
58
ps pancytopenia - hypocellular BM (aplastic anemia <25%) - hyper cellular BM mc adults
MDS
59
hypocellular BM (aplastic anemia <25%)
MDS | PNH
60
megaloblastic anemia infections auoimmune
hypercellular in MDS PNH M/C
61
PNH usually progresses to?
aplastic anemia
62
lap score when AA progresses to PNH?
decreases
63
all are preleukemic except | PCH!
- pnh - mds - aa
64
AA can progress to all except | - PRCA
- pnh - mds - aml
65
AA can become PRCA?
NO
66
MDS types
``` RCUD RCMD RARS RAEB MDS + isolated 5q del ```
67
refractory cytopenias
MDS | do not respond to usual Rx
68
abN in 1 lineage RBC - ref anemia - ref leucopenia - ref thrombocytopenia
>10% of 1 lineage is AbN RCUD ref cytopenia f unilineage dysplasia
69
> 1 lineage affected | > 10% of each cell line abN
RCMD | ref cytopenia of multilineage dysplasia
70
erythroid stains + PRUSSIAN BLUE (PEARLS stain) >15% erythroid cells RINGED SIDEROBLASTS
RARS | ref anemia with ringed sideroblasts
71
PS vs BM <5% 5-9% 5-19% 10-19%
RAEB 1 RAEB 2 ref anemia with excess blasts
72
mc cytogenetic abN in MDS good px PS - thrombocytosis
MDS with isolated 5q del
73
mc cytogenetic abN MDS overall
5q del
74
mc cytogenetic abN MDS adults
5q del
75
mc cytogenetic abN MDS children
monosomy 17 | POOR PROGNOSIS
76
ALL B T
BALL mc 85-90% | TALL 10-15%
77
BALL good Px
1-10y t(12,21) HYPER diploidy
78
BALL poor Px
``` high TLC high blasts % LN + HSM <1y or >10y males testis+ CNS + t(9;22) p190 bcr abl infantile leukemia hypodiploidy ```
79
infantile leukemia
mc t(4;11)
80
ALL FAB classfn
L1 same size of blasts POORER PX L2 varying sizes L3 BURKITTS
81
Most sig Px marker in ALL
minimal residual dis | Assess chemoRx response
82
AML types
M0-7
83
mc AML | lc AML
mc - M2 | lc - M7
84
M7
acute megakaryocytic CD 41 61
85
MPO -ve AML
M0, 6, 7
86
M6
acute ERYTHROLEUKEMIC CD 36, 71 glycophorin A +ve
87
best Px AML
M3
88
M3
acute promyelocytic mc t(15;17) PML RARA CVS # - NBTE
89
PML RARA Rx
ATRA all trans retinoic acid mc COD - DIC
90
DIC mc in which AML
M3
91
M4
acute myelomonocytic
92
M5
acute mnocytic
93
NSE +ve CD 14 16
monocytic leukemias | M4&5
94
mc leukemia ass with DOWNS
ALL - M7
95
mc AML ass with - chloromas - BM fibrosis - leukemia cutis
- chloromas 2 - BM fibrosis 7 - leukemia cutis 5
96
good Px in AML
t(15;17) PML RARA M3 t(8;21) AML ETO/RUNX1- RUNXITI M2 inv 16 M4eo
97
16% of blasts in BM which are - AML ETO+ve - AML ETO -ve
- AML ETO+ve M2 | - AML ETO -ve MDS RAEB2
98
hodkins mc REAL WHO classfn
- classical | - lymphocyte predom
99
mixed cellularity mc INDIA nodular sclerosis mc WEST mc females lymphocyte rich L depleted WORST Px
classical hodgkins
100
best Px hodgkins
lymphocyte predominant hodgins
101
RS reed steenberg cells in reactive background | OWL EYE ap
PS of hodgkins
102
CD 15 30 except in lymphocyte predom?
Hodgkins lymphocyte predom = 15- , 30- , 20+
103
variant of RS cells
- popcorn | - lacunar
104
L&H cell
popcorn cell lymphocyte and histiocytic cell LYPMPHO PREDOM
105
lacunar cell
Nodular sclerosis
106
HL shows bomodal age
- young | - >45y
107
Ann arbor
HL 1. single LN 2. >1LN same side of diaph 3. >1LN on opp side of diaph 4. BM +
108
mc NHL
B NHL MATURE
109
- SLL/CLL - Follicular lymphoma - mantle cell lymphoma - marginal cell lymphoma
mature B NHL | CD 19+ve
110
5+ , 23+ , 200+ | 19+
small lymphocytic lymphoma chronic lymphocytic lymphoma SLL /CLL
111
5- , 10+ mc t(14;18) bcl2+
follicular lymphoma
112
5+, 10-, 23- mc t(11,14) bcl 1+ cyclin D1 +ve
mantle cell lymphoma
113
cyclin D1 -ve Mantle
SOX 11 +ve
114
5-, 10-
marginal cell lymphoma
115
mc NS - adults - >40y
- adults FSGS | - >40y MGN (west - FSGS only)
116
mc NHL in INDIA
DLBCL bcl 6 +ve (west - follicular)
117
mc HL ass with EBV
mixed cellularity
118
mc EBV -ve HL
lymphocyte predom
119
mc HL with HIV
lypmphocyte depleted HL increased incidence BUT | mc - MIXED CELLULARITY
120
high TLC with mature ap lymphocytes smudge cells mc characteristic RBC (SPHAEROCYTES - warm AIHA)
SLL/CLL
121
Px markers of SLL/CLL | GOOD
- IgVH hypermutation (PCR) surrogate: ZAP 70 protein expression - ZAP 70 +ve BAD - mc cytogenetics - NORMAL mc cyto abN del 13q GOOD - BAD Px if Del in 11 and 17
122
burkitt types
- african - non endemic or sporadia - hiv
123
80-90% ebv + mc mandible mc t(8;14) others (2;8) and (8;22)
african or endemic burkitt
124
20-30% ebv ileocecal region c myc protoonc mutation
non endemic burkitt
125
30-40% ebv LN + n myc ass with neuroblastoma
HIV ass burkitt
126
classical triad 19+ 34- sIgM +
mature B cell neoplasm
127
starry sky LN
stars - macrophages | sky - burkitt cells
128
large cells with blue cyto baso cyto cyto vacuoles
PS and BMA in burkitts
129
- inc PLASMA with any 1 of CRAB - plasma >60% in absence of CRAB - mc IgG IL 6 overexpression - M band on electrophoresis - large cell with eccentric nucleus - PS: roloulaux STACK OF COINS, >20% plasma cells
MM
130
normal MCV
80-100
131
immature RBC in PS | indicator of marrow erythropoesis
Reticulocyts | Normal - 0.5 - 2.5%
132
supravital stain mc methylene blue brilliant cresyl blue
retic
133
CRC
Retic count x Hb of pt / normal Hb
134
low retic count
- aplastic anemia - nutritional anemia - CKD - ACD - Myelofibrosis
135
high retic
- hemolytic anemia | - nutritional anemia on Rx PEAKS ON DAY 7
136
microcytic hypochromic
- IDA mc - thal sideroblastic - ACD rarely
137
nromocytic normochromic
``` HIGH retic - hemolytic anemia LOW retic - 1. aplastic 2. ACD 3. CKD 4. myelofibrosis ```
138
macrocytic
megaloblastic 9+12 | non megalo - liver dis
139
mc cause of anemia in hosp pt
ACD
140
mc anemia in ACD
normocytic normochromic
141
nucleo cyto async
megalo nucleus - immature cyto - mature MYELOCYTES & METAMYELOCYTES
142
orthochromatic Hb
intermediate normoblast
143
1st feature on PS in megalo
HS neutrophil - >5lobes in 1 - 5 lobes in 5%
144
anemia classfn on etiology
- blood loss - hemolysis - dec prodn
145
hemolytic anemia causes
- HS mc memb# AD - G6PD mc enz# XR - Hb pathies
146
Hb pathies
- sickle cell AR - thal AR - PNH acquired - autoimmune hemolytic acquired
147
dec prodn of rbc
``` nutrition ACD CKD MF AA ```
148
AD | mc rbc memb# ANKYRIN
HS
149
``` transmemb anion transporter (BAND3) protein 4.2 (PALLIDIN) ankyrin beta spectrin alpha spectrin ```
outer to inner
150
HS most severe defect
ALPHA SPECTRIN
151
reduced deformability by sphaerocyte splenic entrapment HEMOLYSIS extravas
HS
152
RBC + vary conc NS
osmotic fragility testing | - SHIFT TO RIGHT in normal signmoid curve
153
osmotic fragility testing | - SHIFT TO RIGHT
HS | AIHA
154
osmotic fragility testing | - SHIFT TO LEFT
SICKLE CELL IDA THAL
155
``` oxidative damage to globin chain chain ppts in RBC HEINZ bodies formed BITE cells high retic count ```
G6PD beingn self limiting
156
site of hemolysis in g6pd
intra and extra vas
157
intra and extra vas
``` g6pd sickle cell (spleen) autoimmune COLD Ab (liver) ```
158
mc clinically sig variant of gdpd def
mediterranean type most severe low t1/2
159
g6pd test
methHb reduction test
160
point mutation in 6th codon of beta chain Glu acid ---> Valine polymer on deO2 Irreversibly sickled later
sickle cell anemia
161
vaso occlusive crisis splenic changes + inc risk of infections aplastic crisis
sickle cell anemia
162
``` dactilitis of bones of hand and feet stroke retinopathy conj vessels COMMA SIGN priapism ```
vaso occlusive crisis
163
splenic changes + sickle cell
1st change - spleen enlarged multiple ifarcts autosplenectomy
164
H influenza Strep pneumonia mc OM in sicle cell - salmonella
inc risk of infections
165
SPLENECTOMY INDICATED in HS? Sickle cell?
HS YES! | Sickle cell NOOO
166
aplastic crisis in sickle cell
Parvovirus B 19 | erythroid precursor in BM #
167
screening test of choice in sickle cell
Hb Electrophoresis
168
IOC for Hb pathies
HPLC | - quantitative test
169
normal Hb
A a2b2: 88-95% A2 a2d2: 2-3.5% F a2g2: <1%
170
main indication for HbElecrophoresis
screenignof woman by end of 1st trimester for thal minor
171
thal minor HbElectro
HbA2 >3.5% (but <8%)
172
run sample on agar Hb -ve charge cathode - Sample - A2 - S - F - A - anode
HbElecrophoresis
173
Hb migrating to A 2 region
A2 C E mc
174
Hb migrating to S region
S D punjab G
175
what is motility of S compared to Hb A?
reduced - size dep - charge dep - NOT CONC DEPENDENT!
176
dec alpha chains | GENE DELETIONS
alpha thal
177
de beta chains | POINT MUTATION
beta thal | A decreased, F increased
178
HbH b4 | Hb barts g4
physio useless
179
AR Heterozygous MINOR homozygoous MAJOR
beta thal | 2 chains
180
``` 4 chains - del 1 chain 2 3 4 ```
1 chain - asymp carrier 2 - thal MINOR 3 - HbH 4 - Hydrops fetalis
181
Mentzer index
MCV/ RBC count 13
182
hypochromia f/b microcytosis
thal minor
183
microcytosis f/b hypochromia
IDA
184
RDW normal
11.5-14.5%
185
RDW increases
IDA | normal in thal minor!
186
CD 55 DAF decay accelerating factor stops action of C3 convertase
CD 59 MIRL memb inhibitor of reactive lysis stops MAC c5b-9
187
PNH
def of CD 55 and <<< 59 (hemolysis)
188
``` acq mutation in PIGA gene on X Ch def of Gp1 linked anchor protein ```
acq clonal stem cell NOT AUTOIMMUNE coombs -ve
189
mc mortality in pnh
thrombosis | - unusual sites Portal v, hepativ v , cerebral vein BUDD CHIARI
190
tests for pnh
- hams (acid serum lysis) - sucrose lysis ( more sens) - mc GEL CARD (red ring NOZRMAL ) - flow cytometry - FLAER fluroscent pro serolysin test (most specific for pnh f/b flow cytometry
191
IgG AIHA
warm may or may not fix complement 37 deg EXTRAVAS HEMO (SPLEEN)
192
IgM AIHA
``` cold fixes complement 4 deg BOTH EXTRA mc and @liver AND INTRA mycoplasma pneumonia + ```
193
ass with CLL shift to right Ag: BAND 3
warm AIHA
194
tests for AIHA
coombs - direct (Ab on RBC surface) - indirect
195
pt blood + Anti human globin = agglutintn
direct coombs
196
Ab in serum of pt + Sheep rbc + AHG = Agglutn
indirect coombs
197
PS with sphaerocyte next inv
coombs
198
IgG (exception to rule) Donath landsteiner Ab p blod gp Ag
PCH
199
burr cells
ECHINOCYTES spicules on rbc - eq size and eq spaced mc artifact
200
spur cells/ acanthocytes
spicules uneq lenth and spaced | LIVER DIS ABETALIPO
201
bite cells
g6pd def
202
myelofibrosis
tear drop
203
howell jolly | cabot ring
megaloblastic anemia post splenectomy thal
204
pappenheimer bodies
sideroblastic anemia
205
sphaerocyites
HS | AIHA
206
most alarming hemolysis
``` schistocytes MAHA DIC HUS - SHIGA TOXIN 0157:H7 TTP - ADAM TS 1 def HELLP march Hburia def of complement factors BHI prosthetic heart valves ```
207
manual Platelet count
false low due to clumping
208
falciparum RBC malairae vivax
banana rbc left complete rbc destroyed
209
severe anisopoikilo microhpochromic target cells no inc rbc
thal MAJOR
210
basophilic stippling
Pb poisoning thal megalo
211
stages of IDA
1. dec stores 2. Fe def erythropoeisis 3. MCHC
212
Stage 1 IDA
``` BM iron DEC S ferritin DEC (most sens) TIBC INC S Fe NORMAL %transferrin sat NORMAL PS NCNC ```
213
Stage 2 IDA
S Fe DEC %transferrin sat DEC PS NCNC
214
Stage 3 IDA
PS MCHC
215
thrombocytosis
secondary to IDA
216
megalo
most characteristic - pancytopenia | low retic count
217
after rx of megalo
- BM changes become Normal in 24h - PC normal in 1 wk - last to disappear are HS neutrophils 2 wks
218
mc dis causing ACD
- TB - typhoid - hiv - autoimmune - CTD
219
dis NOT ASS with ACD
- CKD anemia - anemia in CLD - worm infestation - any cause of chronic blood loss
220
ACD patho
- inc HEPCIDIN block - cytokines and chemokines suppressing erythropoesis - reduced rbc survival 60-80 days