Wbc Disorders

Front 1

fx of reactiv neutrophilia

Back 1

toxic granulation, vacuolization and dohle bodies

Front 2

fx of MPD

Back 2

persistent neutrophilia, basophilia, lack of toxic changes, myelocyte bulge, LAP score increased unless CML where decreased

Front 3

fx of GM-CSF

Back 3

leukocytosis (prominent toxic granulation)
nRBCs
abnl nuclear segmentation

Front 4

morphologically unremarkable heretidary wbc d/o

Back 4

- Chronic granulomatous disease
– Leukocyte adhesion deficiency
– Myeloperoxidase deficiency

Front 5

gene mutation in chediak-higashi syndrome

Back 5

LYST mutation- defect in lysosome formation
which causes ↓ phagocytosis and
defective microtubule polymerization

defect in granules

Front 6

dz states in chediak-higashi (5)

Back 6

- Abnormal WBC & LGL cause infections
– Melanosomes cause occulocutaneous albinism
– Neurons cause neuropathy
– Platelets cause bleeding
– Accelerated phase, hemophagocytic-like triggered by viral infection (EBV)

Front 7

inclusions in chediak-higashi

Back 7

coarse (large) granules - made of primary and secondary granules

Front 8

what

Back 8

chediak-higashi

Front 9

bottom line chediak-higashi

Back 9

lysomes, LYST, coarse granules, multisystem

Front 10

myh9 related syndromes

Back 10

- May Hegglin Anomaly
– Sebastian Syndrome
– Fletchner Syndrome
– Epstein Syndrome

Front 11

what

Back 11

may-hegglin
made-hegglin (dohle bodies and enlarged plts)

Front 12

myh9 disorder fx

Back 12

getcha early
glomerulonephritis
enlarged plts
thrombocytopenia
cataracts
hearing loss

Front 13

what

Back 13

alder-reilly anomaly

Front 14

what is alder reilly anomaly

Back 14

Dense ametachromatic azurophilic granules in all WBCs, PAS+

"azurophilics retained"

Front 15

specific diseases where see alder-reilly anomaly

Back 15

lysozymal enzymes to break down mucopolysaccharides
– Mucopolysaccharidoses (Hurlers, Hunters)

Front 16

mutations in pelger huet anomaly

Back 16

Autosomal dominant disorder with lamin B receptor mutation

peLger Buet

Front 17

what is gaucher

Back 17

Glucocerebroside Accumulation
Like GAucher

Front 18

what is niemann-pick

Back 18

sphIEngomyelin accumulation

Front 19

hypogranularity of pmn

Back 19

MDS! or understaining :)

Front 20

expression of LGLs

Back 20

CD3+CD16+CD56+

Front 21

what are hematogones and where found; flow results

Back 21

recovering marrow, peds cases
maturing B cell
CD20, CD10, CD34, Tdt+

Front 22

how distinguish between ALL and hematogones on flow

Back 22

hematogones show a range of maturation (both those with CD10 expression and those without); in ALL, all have CD10 expression!!!

Front 23

hantavirus pulmonary syndrome

Back 23

Hb concentration, pulmonary edema, increase immunoblastic morphology, lack of toxic granulation,

Front 24

who gets persisent polyclonal B lymphocytosis

Back 24

female smokers, HLA-Dr7

Front 25

pertussis findings in peripheral smear

Back 25

clefted lymph nuclei, reider cells

Front 26

monocytosis

Back 26

molting monocytes
malaria, listeria, tb

Front 27

if see monocytes and promonocytes and splenomegaly, what should you think

Back 27

neoplasm (monocytic

Front 28

cytokine relatively specific for eosinophils

Back 28

IL5

Front 29

what is loeffler pneumonia

Back 29

eosinophilic pneumonia

Front 30

what is churg-strauss syndrome

Back 30

eosinophilic vasculitis

Front 31

three fx that distinguish MDS

Back 31

ineffective hematopoiesis (with a frequently hypercellular marrow)
cytopenias
often see dyspoeisis in myeloid lineage

Front 32

if see gingival hyperplasia, what should you think of

Back 32

monocytic leukemia

Front 33

what are auer rods

Back 33

fusion of primary (azurophilic) granules

Front 34

exceptions to 20% rule for blasts

Back 34

1. M6 - can be made below this threshold
2. certain cytogenetic findings (e.g. t(8;21)
3. Monoblastic leukemias (can include promyelocytes and promonocytes)

Front 35

in counts, how many are "enough" to count to get blast in marrow and PB

Back 35

marrow - 500
blood - 200

Front 36

four most common immunophenotypic markers for AML

Back 36

HLA-DR (not seen on APML)
CD13,
CD33
CD45

usu do not see lymphoid markers but can see 20% have CD7

Front 37

shared fx of AML with t(8;21) and inv (16)

Back 37

- younger patients
- good chemo response
- both translocations involve a chain of core binding factor (CBF) - 8;21 has AML1 gene which is CBFa
the inv 16 involves CBFb

Front 38

morphology of 8:21 blasts (and Ihc)

Back 38

M2: so AML with maturation; pronounced azurophilic granularity, having large granules and Auer rods

so really interesting ihc that could alone s/o this diagnosis: CD34 (early marker) and aberrant CD15 (late marker)
others: CD34, CD13, CD33 and HLA-Dr (as expect)
if have CD56, worse prognosis

Front 39

monocytic markers (6!)

Back 39

CD4, CD11b, CD14, CD24, CD64, CD68

Front 40

t(8;21) genes

Back 40

AML1/CBFa-ETO (aka: RUNX1/RUNX1T1)

Front 41

inv(16) genes

Back 41

CBFB-MYH11

Front 42

less common AML with recurrent translocations
t(9;11) genes
t(6;9) genes
inv3
aml with mutated npm1
aml with mutated cebpa

Back 42

t(9;11) genes: mllt3-mll3
t(6;9) genes: DEK-NUP214
inv 3 RPN1:EV1

Front 43

erythroid markers

Back 43

glycophorin and hemoglobin A

Front 44

megakaryocytic markers

Back 44

CD42 (gpIb/IX)
CD41 (gpIIb/IIIa)
CD61 (gpIIIa),
CD36 (gpIIIb/IV)

Front 45

what

Back 45

block positivity for lymphoblasts with PAS

Front 46

three histochemical stains for myeloid markers

Back 46

-MPO (deteriorates with time)
- NSE (two: alpha napthyl esterase, alpha napthyl butyrate)
- sudan black B (marks lipid)

Front 47

strict criteria for M4

Back 47

– >20% blasts including
promonocytes
– >20% neutrophils and precursors
– >20% monocytes and precursors

Front 48

strict criteria for M5 (acute monoblastic and monocytic leukemia)

Back 48

>80% of cells are monocytic cells
– M5a-- >80% of monocytic cells are monoblasts
– M5b-- <80% of monocytic cells are monoblasts

Front 49

strict criteria for M6

Back 49

Erythroleukemia M6a
> 50% of all nucleated cells erythroid cells
• >20% of non-erythroid cells are myeloblasts
Pure erythroid leukemia M6b (rare)
– >80% erythroblasts

Front 50

strict criteria for M7

Back 50

> 20% blasts, 50% are megakaryoblasts

Front 51

what, with M7 morphology, is excluded from M7 diagnosis

Back 51

Excludes t(1;22), inv(3), t(3;3) and Down syndrome-related cases

Front 52

of the three common aml cytogenetics, order them in prognosis

Back 52

t(15;17) best
t(8;21)
inv(16)

Front 53

strict criteria for M3

Back 53

> 20% (blasts + abnormal promyelocytes)

Front 54

what are the variant APML and why do we care

Back 54

resistant to ATRA
- T(5;17)(q23;q12) • 5 – nucleophosmin gene
– t(11;17)(q13;q21) • 11- nuclear matrix association
– t(11;17)(q23;q21) • 11 – ZBTB16 (previously PLZF)

Front 55

prognosis

Back 55

good: APML, microgranular variant
butterflies

Front 56

pertinent fx in t(9;11)(p22q23) AML

Back 56

involves MLL
kids
monocytic differentiation
often get secondary abnormalities (ex. trisomy 8)

Front 57

details on t(6;9)(p23q34) AML

Back 57

basophilia
poor prognosis
DEK-NUP214

Front 58

more on inv(3)

Back 58

RPM1-EVI1
• Dysplastic small hypolobated megakaryocytes

Front 59

more on t(1;22)

Back 59

RBM15-MLK1
• Restricted to infants and children <3 years old
• Megakaryoblasts

Front 60

name three alkylating agents

Back 60

Busulfan, chlormabucil, cyclophosphamide

Front 61

name some topo II inhibitors

Back 61

Epipodophillotoxins,
anthracyclins

Front 62

timing and prognosis of aml due to alk agents

Back 62

5-6 years, poor

Front 63

timing and prog of topo II inhibitors

Back 63

2-3 years, not related to MDS, better prognosis
often monocytic

Front 64

various conclusions about aml mutations NPM1 and CEBPA

Back 64

NPM1 mutation with concurrent FLT-3 wild-type
status is prognostically favorable.
• NPM1 mutation with concurrent FLT-3 mutation is
not prognostically favorable
• CEBPA mutation is prognostically favorable

Front 65

B-ALL phenotypic markers

Back 65

• Blasts, TdT and CD34
• B markers: CD19 usually +, CD20 often –
• CD10+/-
• Surface immunoglobulin usually negative

Front 66

special phenotypic findings with t(4;11) B-ALL

Back 66

T(4;11) CD10-, CD15+

Front 67

special phenotypic markers with T(1;19)

Back 67

CD10+, CD34+ ALL

Front 68

what

Back 68

b cell development

Front 69

staining for PAX - pattern

Back 69

nuclear

Front 70

staining for Tdt

Back 70

nuclear

Front 71

staining for CD10

Back 71

membranous

Front 72

staining for CD34

Back 72

membranous

Front 73

seven (!) B ALL with recurrent translocations

Back 73

B LBL w t(9;22)(q34;q11.2); BCR-ABL1
2. B LBL w t(v;11q23); MLL rearranged
3. B LBL w t(12;21)(p13;q22);TV6/RUNX1
4. B LBL w hyperdiploidy
5. B LBL w hypodiploidy
6. B LBL w t(5;14)(q31;q32);IL3-IGH
Assoc with eosinophilia
7. B LBL w t(1;19)(q23;p13.3)E2A-PBX1

Front 74

three good prognostic indicators for BALL

Back 74

- t(12;21) or ETV6-RUNX6 fusion
• Hyperdiploid > 50
• Age 1-10

Front 75

5 poor prognostic factors in B-ALL (3 specific)

Back 75

- Hypodiploid
• t(9:22); BCR/ABL
• 11q23; MLL; t(4;11) MLL/AF4 fusion
• RUNX1 amplification
• Complex abnormalities

Front 76

infant B-ALL

Back 76

4-11; "inf"ormation

Front 77

child ALL translocation

Back 77

t(12;21)

Front 78

adult B-ALL translocation

Back 78

t(9;22)

Front 79

T-all location and lab findings

Back 79

mediastinal mass, hypercalcemia

Front 80

B-ALL phenotype

Back 80

CD34, HLA-DR, CD99
Tdt,
CD19
often can have dim myeloid ag - don't let that deter you!

Front 81

ALL with t(1;19) immunophenotype

Back 81

CD34- CD20-
CD19+, CD9+, CD10+

Front 82

precursory T ALL immunophenotype

Back 82

CD34+, CD99+
tdt+
CD7+ (important)
CD2, CD3, CD5 (either double positive or double neg CD4/8)
note: neg for HLA-DR

Front 83

progn for 1;19

Back 83

bad

Front 84

special immunophenotypic markers and cytogenetics for infant type ALL

Back 84

t(4;11); CD15+, lack of CD10

Front 85

what is one of the most specific markers for myeloid derivation

Back 85

CD117

Front 86

some mutations in T-ALL

Back 86

affect czomes 7 (BG) and 14 (AD) where tcr are

some partner genes:
Myc (8q24)
– TAL1(1q32)
– RBTN1(11p15), RBTN2 (11p13)
– HOX11(10q24)

Front 87

mutations in PV

Back 87

this is the really significant JAK2 dz
Jak2 (V617F) - most and JAk2 (exon 12)
also Tet2 (16%) and IDH1/2 (a/w blast transformation)

Front 88

peripheral blood findings for PV

Back 88

-increased rbcs (hct >60%), hb>18.5m, >16.5f, inc mcv
-panymyelosis (increased plts, wbcs)
- teardrop rbcs

(note in BM: hypercellular, megas are big)

Front 89

fx of ET

Back 89

most common MPN
- largely restric to megas/plts (large mega, increased plts to >450/600)
hb<13

Front 90

stains for ET

Back 90

CD61 (gbIIIa)
and CD64

Front 91

mutations for ET

Back 91

jak2 and mpl

Front 92

which is the bad myeloproliferative d/o

Back 92

primary myelofibrosis

Front 93

findings in primary MF

Back 93

teardrop rbcs
panmyelosis
more mega "atypia"
inreased likelihood of blast transformation
Jak2/mpl

Front 94

another name for teardrop cells

Back 94

dacrocytes

Front 95

cytogenetics in CML

Back 95

bcr-abl,
i(17q), trisomy 8 and 19

if eosinophilia, PDGRA, B and FGFR

Front 96

findings in CML

Back 96

decreased LAP score
wbc>50K
myelocyte bulge
sea blue histiocytes
hypolobated megas
basophilia, eos

Front 97

most common bcr-abl in cml, cnl and all

Back 97

cml- 210
cnl - 230
all - 190

Front 98

cytopenias in mds

Back 98

- Hb <10g/dL, macrocytic anemia
– Neutrophil count <1800/uL
– Platelet count <100,000/uL

Front 99

some cytogenetics in MDS

Back 99

-5, 5q-, -7, +8, 20q-, +9, -18, -19, +21

Front 100

good prognostic markers in MDS

Back 100

Normal, -Y, del(5q), del(20q), TET2 mutations

Front 101

poor prognostic markers in MDS

Back 101

Complex (>3 abnormalities) or chr 7
abnormalities

Front 102

flow testing for MDS

Back 102

- Coexpression of CD7, CD56, CD10, CD11b or CD15 on blasts
- Absence of CD33, CD13 or HLADR on blasts
- Abnormal CD56, CD19, CD7 or CD5 on myeloid cells
- hypogranularity of PMNs
- loss of CD13/16 maturation (nike swoop)

Front 103

findings in mds with isolated 5q-

Back 103

Hypolated megas & thrombosis
– RPD14 deficiency,

Front 104

cell lines most affect in childhood refractory cytopenia

Back 104

-neutropenia and thrombocytopenia

Front 105

JMMoL

Back 105

Pediatric MPD/MDS
• Poor prognosis
• increased HbF
• increased sensitivity to GM-CSF
• RAS/NF1/CBL/PTPN11 mutations

Front 106

who has an increase in JMMoL

Back 106

• ↑ in patients with neurofibromatosis 1 or
Noonan syndrome