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Heme II > Neoplasms of white cells > Flashcards

Neoplasms of white cells Flashcards

1
Q

inherited genetic risk factors for leukemia

A 
bloom syndrome
fanconi anemia
ataxia telangiectasia
down syndrome
Type I neurofibromatosis
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2
Q

Viruses and leukemias

A

HTLV-1
EBV
KSHV/HHV-8

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3
Q

HTLV-1

A

adult T cell lymphoma

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4
Q

EBV

A 
burkitt lymphoma
HL
B-cell lymphomas
rare NK cell lymphomas
(benign HLH)
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5
Q

chronic inflammation that can lead to CA

A

H. pylori
gluten sensitive enteropathy
breast implants
HIV

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6
Q

H. pylori

A

gastric B cell lymphomas

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7
Q

gluten

A

intestinal T cell lymphomas

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8
Q

breast implants

A

T cell lymphomas

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9
Q

HIV

A

B cell lymphomas

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10
Q

smoking

A

acute myeloid leukemia

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11
Q

Primary T cell associated Ags

A 
CD1
CD3
CD4
CD5
CD8
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12
Q

Primary pre-B cell associated Ags

A

CD10
CD19
CD20

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13
Q

primary mature B cell associated Ags

A

CD19
CD20
CD21
CD23 (activated B cells)

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14
Q

CD21

A

EBV R

and on DCs

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15
Q

CD23

A

only activated B cells

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16
Q

CD11c

A

hairy cell leukemia

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17
Q

CD15

A

RS cells and variants

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18
Q

CD30

A

RS cells and variants

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19
Q

precursor B cell neoplasms

A

B-ALL

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20
Q

precursor T cell neoplasms

A

T-ALL

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21
Q

B-ALL

A 
childhood acute leukemia (uncommon presents in bone or skin)
most common cancer in children
W:B 3:1
slightly more frequent in boys
hispanics highest incidence
peaks at age 3
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22
Q

T-ALL

A

less common
adolescent males as thymic lymphomas
evolve to leukemic picture

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23
Q

B-ALL mutations

A

loss of fnx in PAX5, E2A, EBF, ETV6, RUNX1

12;21

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24
Q

T-ALL mutations

A

gain of fnx in NOTCH1

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25
Q

ALL mutations

A

90% have numerical or structural chromosome changes

most commonly hyperploidy

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26
Q

ALL morphology

A

marrow hypercellular
mediastinal thymic masses
lymphadenopathy
macriphages have stary sky appearance

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27
Q

B-ALL immunophenotype

A 
CD19
PAX5
CD10 +/-
CD20
IgM heavy chain
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28
Q

T-ALL immunophenotype

A

CD1- 5

CD7 and 8

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29
Q

clinical ALL

A

Abrupt stormy onset w/in days to weeks

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30
Q

symptoms of ALL due to bone marrow supression

A

anemia
low grade fever
infections
bleeding

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31
Q

other symptoms of ALL

A 
bone pain
generalized lymphadenpathy
splenomegaly
hepatomegaly
testicular involvement
meningeal (B-ALL)
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32
Q

worse prognosis ALL

A

100,000

(9;22)- Philadelphia chrom

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33
Q

favorable prognosis ALL

A 
2-10 yrs
low white count
hyperdiploidy
trisomy of 4,7,10
presence of t(12;21)
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34
Q

CLL

A 
chronic lymphocytic leukemia
absolute lympohocytosis >5000
most common leukemia of adults in western world
median age 60
less common in japan and asia
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35
Q

CLL mutations

A 
deletion of:
13q14.3
11q
17p
trisomy 12q
gain of fnx NOTCH1R in 18%
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36
Q

CLL pathogenesis

A

growth limited to proliferation centers due to stromal cells TFs
unmutated Ig segments mor aggressive

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37
Q

CLL morphology

A

proliferation centers- larger activated lymphocytes gathered in loose aggregates
pathognomonic for CLL
smudge cells
infiltrates in splenic white and red pulp and portal triads

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38
Q

CLL immunophenotype

A 
CD19
CD20
CD23
CD5
low level expression of surface Ig
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39
Q

CLL presentation

A

usually asymptomatic at Dx

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40
Q

CLL symptoms

A 
fatigability
weight loss
anorexia
lymphadenopathy
hepatosplenomegaly
small monoclonal Ig spike
infections
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41
Q

CLL and immune fnx

A

hypogammoglobulinemia common
increased susceptibility to bacterial infections
hemolytic anemia
thrombocytopenia

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42
Q

worse prognosis CLL

A

deletion of 11q and 17p
lack of somatic hypermutations
ZAP-70
NOTCH1 mutations

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43
Q

richter syndrome

A

occurs when CA transforms to more aggressive DLBCL
rapidly enlarging mass w/in lymph nodes or spleen
survival <1yr

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44
Q

follicular lymphoma

A

most common form of indolent NHL in US

less common in Europe, rare in asia

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45
Q

follicular lymphoma pathogenesis

A

hallmark is (14;18) -> overexpression of BCL2
BCL2 antagonizes apoptosis and promotes survival
MML2 mutations -> epigenetics

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46
Q

follicular lymphoma morphology

A

predominately nodular or nodular and diffuse
no clear germinal centers visible
lymphocytosis in 10%
bone marrow involvement in 85% -> paratrabecular lymphoid aggregates
splenic white pulp and hepatic portal tirads frequently involved

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47
Q

Follicular lymphoma immunophenotype

A 
CD19
CD20
CD10
Surface Ig
BCL6
BCL2
unlike CLL CD5 negative
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48
Q

Follicular lymphoma clinical

A 
generalized lymphadenopathy
extranodal sites uncommon
waxing and waning course
incurable anti CD20 may help
histologic transformation in 30-50% most commonly to DLBCL survival <1yr
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49
Q

DLBCL

A

diffuse large B cell lymphoma
most common NHL in US
slight male predominance
median age 60

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50
Q

DLBCL pathogenesis

A

dysregulation of BCL6
may arise from follicular cell lymphomas
5% MYC
epigenetics

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51
Q

DLBCL morphology

A

large cell size
diffuse pattern, no follicular formations
large mutlilobar or cleaved nuclei
karyorexis

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52
Q

DLBCL immunophenotype

A

CD19
CD20
+/- CD10, BCL5

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53
Q

DLBCL subtypes

A

immunodeficiency associated

primary effusion lymphoma

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54
Q

immunodeficiency DLBCL

A

HIV

EBV

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55
Q

primary effusion lymphoma DLBCL

A

always infected w/KSHV/HHV-8
typically do not express B or T cell markers
have clonal IgH

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56
Q

DLBCL clinical

A

rapidly enlarging mass at nodal or extranodal site
waldyer ring
liver and spleen large destructive masses
bone marrow rarely involved
aggressive and rapidly fatal w/o Tx
MYC worse prognosis
if circulating neoplastic B cells worse prognosis

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57
Q

Burkitt lymphoma types

A

african/endemic/mandibular
sporadic/nonendemic
aggressive lymphomas in HIV

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58
Q

Burkitt pathogenesis

A 
MYC on chrom 8
Warburg effect
fastest growing CA
sporadic usually class switch regions
essentially all endemic have EBV
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59
Q

Burkitt morphology

A

diffuse infiltration
phagocytes have starry sky pattern
royal blue cytoplasm w/clear cytoplasmic vacules

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60
Q

Burkitt immunophenotype

A 
IgM
CD19
CD20
CD10
BCL6
consistent w/germinal center B cell origin
almost always neg for BCL2
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61
Q

Burkitt clinical

A

children and young adults
endemic- mandible
sporadic- GI
worse prognosis in older adults

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62
Q

solitary myeloma

A

MM infrequent variant
presents as single mass in bone or soft tissue
soft tissue can usually be cured w/excision

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63
Q

smoldering myeloma

A

MM uncommon variant defined by lack of symptoms and elvelated plasma M, but < 3

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64
Q

Waldenstom macroglobinemia

A

syndrome in which high IgM leads to hyperviscosity symptoms
usually older adults
associated w/lymphoplasmacytic lymphoma

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65
Q

MM

A

commonly associated w/lytic bone lesions, hypercalcemia, renal failure, and acquired immune abnormalities
higher incidence in men and african descent
peak 65-70

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66
Q

MM pathogenesis

A 
cyclin D1
deletions of 17p (TP53gene) -> poor prognosis
late state disease MYC rearrangments
NFkB mutations
High level of IL6 poor prognosis
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67
Q

definition of MM

A

> 3 serum Ig (IgG most common)
and/or
6 BJ proteins

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68
Q

plamacytomas

A

destructive plasma cell tumors of axial skeleton seen in MM

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69
Q

Bone lesions on radiograph

A

appear as punched out defects consisting of soft gelatinous red tumor masses

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70
Q

malignant plasma cells

A

like their benign counter parts, have perinuclear clearing
can be relatively normal appearing blast cells
or
bizarre multinucleated cells

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71
Q

buzz words for MM

A 
flame cells
mott cells
russel bodies
sitcher bodies
rouleaux formation
plasma cell leukemia
meyloma tumor
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72
Q

flame cells

A

fiery red cytoplasm

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73
Q

Mott cells

A

multiple grape like cytoplasmic droplets

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74
Q

MM cell inclusions

A

fibrisl
crystaline rods
globules

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75
Q

russel bodies

A

globular inclusions in cytoplasm

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76
Q

Ditcher bodies

A

globular inclusions in nucleus

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77
Q

rouleaux formations

A

high levels of M proteins cause red cells to stick together

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78
Q

plasma cell leukemia

A

rare, when tumor cells enter peripheral circulation

79
Q

MM immunophenotype

A

CD138 (aka syndecan-1)

often express CD56

80
Q

Symptoms of MM stem from

A

effects of plasma cell growth in tissues (bones)
production of excessive Igs
suppression of normal humoral immunity

81
Q

symptoms of MM

A

bone pain and fragility
decreased normal Igs -> recurrent bacterial infections
amyloodosis
renal insufficiency (major COD)

82
Q

better prognosis MM

A

translocations of cyclin C1

83
Q

worse prognosis MM

A

deletions of 13q, 17p, (4;4)

84
Q

MGUS

A

asymptomatic
serum M protein <3
1% develop symptomatic MM per year
same genetic markers as MM, therefore thought to be early stage

85
Q

lymphoplasmacytic lymphoma

A

superficial resemblance to CLL/SLL (but tumor cells are plasma)
secretes IgM causing hyperviscosity syndrome -> waldenstom macroglobulinemia
unlike MM light chains are rare and no bone lesions

86
Q

lymphoplasmacytic lymphoma pathogenesis

A

acquired mutations in MYD88

87
Q

lymphoplasmacytic lymphoma morphology

A

russel bodies

dutcher bodies

88
Q

lymphoplasmacytic lymphoma immunophenotype

A

CD20

IgM

89
Q

clinical lymphoplasmacytic lymphoma

A 
hepatosplenomegaly
anemia common
10% autoimmune hemolysis due to cold agglutinins 
hyper-viscosity syndrome
incurable progressive disease
plasmopheresis can help
Anti CD20
90
Q

Mantle cell lymphoma

A

male predominance

resemble normal mantle zone B cells

91
Q

Mantle cell pathogenesis

A

almost all have (11;14) -> overexpress cyclin D1

92
Q

mantle cell morphology

A

lymphandenopathy
lymphomatoid polyposis in GI (misdiagnosed as inflammatory bowel disease)
large cells resembling centroblasts and proliferation centers are absent distinguishing from follicular lymphoma and CLL

93
Q

mantle cell immunophenotype

A

CD19
CD20
surface Ig
usually CD5+ and CD23- distinguishing from CLL
IgH lacks somatic hypermutation, therefore naive B cell origin

94
Q

mantle cell clinical

A

most common presentation is painless lymphadenopathy
prognosis poor
blastoid variant even worse prognosis

95
Q

marginal zone lymphomas

A

aka MALTomas
arise w/in GI lymph nodes, spleen, extranodal tissue
memory B cell origin

96
Q

marginal lymphomas inflammatory conditions

A 
sjogren -> salivary gland
hashimotos -> thyroid 
H. pylori -> stomach
may regress if condition treated
lie on continuum btwn reactive and malignant
97
Q

mutations that make marginal lymphomoas Ag independent

A

(11;18)
(14;18)
(1:14)
all of these upregulate BCL10 or MALT1 increasing TFkB

98
Q

hairy cell leukemia

A

rare B cell neoplasm
middle aged white males
M:F 5:1

99
Q

hairy cell leukemia pathogenesis

A

BRAF

100
Q

hairy cell leukemia morphology

A

hair like projections
marrow has diffuse interstitial infilatrate -> dry tap
splenic red pulp usually heavily infiltrated beefy red gross appearacne
hepatic portal triads frequently involoved

101
Q

hairy cell immunophenotype

A 
CD19
CD20
Ig (usually IgG)
CD11c
CD25
CD103
annexin A1
102
Q

hairy cell clinical

A 
splenomegaly often massive
lympadenopathy is rare
pancytopenia
infections (myco)
BRAF inhibitors successful
prognosis excellent
103
Q

peripheral T cell lymphoma unspecified

A 
far less common ten B cell, more common in Asia
mixure of variably sized malignant cells 
prominent inflitrate of reactive cells
brisk neoangiogeneis
CD2, 3, 5
alpha, beta, gamma, or delta receptor
some CD4,8
PRUITIS
worse prognosis then B cell
104
Q

anaplastic large cell lymphoma ALK postive

A

due to ALK fusion proteins -> RAS and JAK/STAT
hallmark cells
ALK in children usually presents in soft tissues better prognosis
ALK negative adult variant worse prognosis
both ALK + and - express CD30

105
Q

Adult T cell leukemia/lymphoma

A

neoplasm of CD4 only in adults

ALL infected with HTLV-1

106
Q

HTLV1 endemic in

A

soutern japan
west africa
caribbean basin

107
Q

common presentation of Adult T cell leukemia/lymphoma

A 
skin lesions
general lymphadeneopathy
hepatosplenomegaly
peripheral blood lymphocytosis
hypercalcemia
clover leaf or flower cells
sometimes progressive demylinating disease of CNS
108
Q

HTVL1

A

encodes TAX -> NFkB

109
Q

Mycosis fungoides

A

tumor of CD4 cells that home to skin

often have cerebriform appearance

110
Q

sezary syndrome

A

variant of Mycosis fungoides
skin involvement is manifested as exfoliative erythroderma
still have cerebriform appearance

111
Q

ceribriform appearance immunophenotype

A

CLA
CCR4
CCR10
all of these home to skin

112
Q

large granular lymphocytic leukemia

A

T cell and NK cell variants of this rare neoplasm occur in adults

113
Q

T cell large granular lymphocytic leukemia

A

mild to moderate lymphocytosis and splenomegaly

lymphadenopathy and hepatomegaly usually abscent

114
Q

large granular lymphocytic leukemia mutations

A

STAT3

115
Q

large granular lymphocytic leukemia tumor cells

A

large lymphocytes w/abundant blue cytoplasm and few course azurophilic granules, best seen in peripheral blood smears

116
Q

large granular lymphocytic leukemia immunophenotype

A

T cell- CD3

NK cell- CD3- CD56+

117
Q

large granular lymphocytic leukemia clinical

A

neutropenia and anemia
rarely pure red cell aplasia seen
felty syndrome triad

118
Q

felty syndrome tirad

A

RA
splenomegaly
neutropenia
underlying autoimmune condition suspected

119
Q

extranodal NK/T cell lymphoma

A

rare in US and europe, more common in asia
destructive nasopharyngeal mass, less common sites are testes and skin
typically surround and invades small vessels -> extensive ischemic necrosis
HIGHLY ASSOCIATED w/EBV

120
Q

extranodal NK/T cell lymphoma immunophenotype

A

CD3- lack T cell Rs rearrangments and express NK cell markers

121
Q

extranodal NK/T cell lymphoma clinical

A

highly aggressive, responds to radiation, but if late in course poor prognosis

122
Q

hodgkin lymphoma

A 
arises in single node or chain of nodes and spreads in predictable pattern
RS cells
2 spikes 20s and 60s
one of most common CAs of young adults
curable in most casses
123
Q

RS cells

A

release cytokines and chemokines and other factors that induce accumulation of reactive immune cells which make up 90% of tumor
usually derived from germinal centers or post germinal center B cells

124
Q

HL classifications

A 
nodular sclerosis
mixed cellularity
lymphocyte rich
lymphocyte depletion
lymphocyte predominance
125
Q

HL pathogeneis

A

Ig genes in RS cells due to epigenetics
usually derived from germinal centers or post germinal center B cells
fail to express most B cell markers including Ig
activation of TF NFkB common in classical

126
Q

RS cells mutations due to

A

EBV
loss of fnx of IkB or A20
copy number gains in REL on chrom 2p

127
Q

RS cell morphology

A

large multiple nuclei or single multilobar nucleus

w/large inclusion like nucleolus

128
Q

mononuclear variant

A

single nucleus w/large inclusion like nucleolus

129
Q

Lunar cell variant

A

seen in nodular sclerosis

more delicate, folded, or multilobate nuclear and pale cytoplasm

130
Q

mummification

A

cell death of RS cells where cell shrinks and becomes pyknotic

131
Q

classical immunophenotype

A

PAX5
CD15
CD30
neg for other B and T cell markers and CD45

132
Q

NODULAR sclerosis type

A 
most common
lacunar variant
deposition of collagen in bands that divide involved lymph nodes into nodules
mediastinal
uncommonly associated w/EBV
excellent prognosis
133
Q

mixed cellular type

A 
diagnostic RS and mononuclear variants usually plentiful
EBV in 70%
M>F
older age
systemic sympotms
overall prognosis good
134
Q

lymphocyte rich type

A

uncommon
reactive lymphocytes vast majority of cells
frequent mononuclear variants and diagnostic RS cells
vague nodularity due t residual B cell follicles
EBV 40%
excellent prognosisj

135
Q

lymphocytes depletion type

A 
least common HL
immunophenotyping essential to differentiate from large cell NHLs
EBV 90%
older adults
HIV of any age
nonindustrialized countries
prognosis less favorable then other HLs
136
Q

lymphocyte predominance type

A 
uncommon 
nonclassical
infiltrate of small lymphocytes and macros
RS hard to find
contains L&H variants
typically nodular pattern of growth
ongoing somatic hypermuations
EBV NOT associated
137
Q

L&H variant

A

multilobar nucleus resembing popcorn kernel
express typical B and T cell marker CD20, BCL6
neg for CD15 and CD30

138
Q

lymphocyte predominance type clinical

A

M>F
<35
present w/cervical or axillary lymphadenopathy
mediastinal and bone marrow involvement rare
may transform to DLBCL
excellent prognosis, but more likely to recur

139
Q

HL clinical

A

cutaneous immune unresponsiveness -> anergy
suppressed TH1 response
spread nodal -> splenic -> hepatic -> marrow and other tissues
tumor stage
anti CD30

140
Q

AML

A

accumulation of immature meloid blasts produces marrow failure and complications related to anemia, thrombocytopenia, and neutropenia
AML all ages, peaks at 60

141
Q

WHO classifications of AML

A

genetic abberations
Post MSD
therapy related
AML, not otherwise specified

142
Q

M0

A

minimally differentiated

myeloperoxidase neg

143
Q

M1

A

without maturation
>3% myeloperoxidase pos
few granules or auer rods

144
Q

M2

A

myelocytic maturation
30-40%
aurer rods
(8;21)

145
Q

M3

A 
acute promyelocytic leukemia
5-10%
may aurer rods patients younger
DIC
(15;17)
146
Q

M4

A

myelomonocytic maturation
myeloperoxidase pos
monoblasts pos for non-specific esterases
inv(16)

147
Q

M5

A 
monocytic maturation
myeloperoxidase neg
esterase pos
mature monocytes inperipheral blood
older patients
high incidence of organomegaly, lymphadenopathy, and tissue inflitrations
148
Q

AML pathogenesis

A

(8;21) (M2) and inv (16) (M4) -> disrupt RUNX1 and CBFB genes
(15;17) (M3) -> PML-RARa fusion protein
activating mutation FLT3
epigenetics

149
Q

AML morphology

A

at least 20% blasts in marrow

150
Q

types of blasts

A

myeloblasts

monoblasts

151
Q

myeloblasts

A

aurer rods

fine peroxidase pos

152
Q

monoblasts

A

lack aurer rods

nonspecific esterase pos

153
Q

AML immunophenotype

A

CD33

CD34

154
Q

AML following MDS or Tx

A

often have deletions in chrom 5&7

155
Q

AML clinical

A

present w/in a few weeks or months on onset of symptoms
spontaneous bleeding (mucosal) or thrombotic events
infections frequent (fungal, psudo)
CNS less common then in ALL

156
Q

meyloblastoma, granulocytic sarcoma, or cholroma

A

occasional presentation of soft tissue mass that w/o Tx will progress to AML

157
Q

MDS

A

maturation defects that are associated w/ineffective hematopoiesis -> cytopenia
blasts <20% in BM
primary (idiopathic) or secondary to drugs or Tx (tMDS)

158
Q

MDS pathogenesis

A 
epigenetics
loss of fnx in TP53
monosomies 5&7
deletions of 5q. 7q, and 20q
trisomy 8
159
Q

MDS morphology

A 
marrow usually hypercellualr at Dx
ring sideroblasts
megaloblastoid maturation
nuclear budding abnormalities
pseudo-pleger-huet cells
meyloid blasts
160
Q

ring sideroblasts

A

erythroblasts w/iron laden mito visible as perinuclear granules in prussian blue stain

161
Q

megaloblastoid maturation

A

resembling that seen in B12 and folate deficiency

162
Q

neutrophils contain

A

toxic granulations and/or Dohle bodies

163
Q

pesudo-pleger-huet cells

A

nuetrophils w/only 2 nuclear lobes

164
Q

myeloid blasts

A

less then 20% of BM

165
Q

MDS peripheral blood

A 
pesudo-pleger-huet cells
giant platelets
macrocytes
poikilocytes
relative or absolute monocytosis
166
Q

MDS clinical

A 
primary is predominately disease of older adults
mean age 70
50% discovered incidently
live 9-29 months
progression to AML in 10-40%
167
Q

MPD

A

common pathogenic feature is presence of mutated constitiutvely activated TK
increases proliferative drive in bone marrow -> increased WBCs
extramedullary hematopoiesis

168
Q

spent phase MPD

A

marrow fibrosis and peripheral blood cytopenias

169
Q

types of MPD

A

CML
PCV
ET
primary meylofibrosis

170
Q

CML

A

chronic myelogenius leukemia

chimeric BCR-ABL gene (Philadelphia chrom) -> good prognosis in CML

171
Q

CML pathogenesis

A

active TK -> RAS and JAK/STAT

172
Q

CML morphology

A

marrow hypercellular w/elevated eos and basophils
megakaryocytes increased, small
sea-blue histiocytes
leukocytosis >100,000
platelets increased
spleen enlarged due to extramedullary hematopoiesis, may infarct

173
Q

sea-blue histocytes

A

scattered macros w/abundant wrinkles

green blue cyto

174
Q

CML clinical

A 
adults
insidious onset
mild-moderate anemia and hypermetabolism
accelerated phase in 50%
blast crisis in 50%
175
Q

PCV

A

Polycythmia vera
point mutation in TK JAK2
increased marrow production of red cells, granulocytes, and platelets

176
Q

PCV pathogenesis

A

erythropoietin levels low
increased blood viscosity
prone to thrombosis and bleeding

177
Q

PCV morphology

A

marrow hypercellular
marked increase in reticulin
mild organomegaly
peripheral blood had increased basophils and large platelets
often progresses to spent phase with extensive marrow fibrosis and prominent organomegaly

178
Q

PCV clincial

A 
veins distended
plethroic and cyanotic
HA
pruitis
hyperuricemia, gout -> phlebotomy
MAJOR RISK OF BLEEDING/THROMBOSES -> COD w/in months w/o Tx
Dx due to DVT, stroke, MI
spent phase bad prognosis
179
Q

ET

A 
essential thrombocytosis
point mutation is JAK2 or MPL 
elevated platelets w/o polycythemia and marrow fibrosis
megakaryocytes often increases in size and number
mild leukocytosis
uncommonly transforms to AML
>60
thrombosis and hemorrhage
Erythromelalgia
gentle chemo
180
Q

Erythromelalgia

A

throbbing and burning of hands and feet do to occlusions of small vessels

181
Q

Primary myelobribrosis

A 
obliterative marrow fibrosis
cytopenias and extensive extramedullary hematopoiesis
identical to spent phase of PCV
activating JAK2 and MPL mutations
PDGF and TGFbeta implicated
severe anemia
182
Q

Primary myelobribrosis morphology

A

erythroid and granulocytic precursors appear normal, but megakaryocytes are large, dysplastic, and clustered
fibrotic marrow may be converted to bone (osteosclerosis)
splenomegaly
leukoerythroblastosis -> release of immature cells
tear drop shaped red cells

183
Q

Primary myelobribrosis clinical

A

spelnomegaly
moderate to seer normochromic normocytc anemia w/leukoerythroblastosis
infections, thrombotic episodes, bleeding, transformation to AML

184
Q

langerhans cell histocytosis mutations

A

activating BRAF
TP53
RAS
TK MET

185
Q

langerhans cell histocytosis morphology

A

birbeck granules

tennis racket like appearance

186
Q

langerhans cell histocytosis immunophenotype

A

HLA-DR
S-100
CD1a

187
Q

langerhans cell histocytosis types

A

mutlifactorial, multisystem (letterer-siwe disease)
unifocal and multifocal unisystem (eosinophilic granuloma)
pulmonary
express CCR6 and CCR7 to home out of skin

188
Q

mutlifactorial, multisystem (letterer-siwe disease)

A 
<2
cutaneous lesions
concurrent hepatosplenomegaly, lymphadenopathy, pulmonary lesions, destructive bone lesion
infection (otitis media and mastoiditis)
rapidly fatal if untreated
189
Q

unifocal and multifocal unisystem (eosinophilic granuloma)

A

langerhans cells w/eos, lymphocytes, plasma cells, neutros

arises in medullary cavity of bones

190
Q

unifocal

A

often in skeleton of older children adults

heal spontaneously or w/excision and radiation

191
Q

multifocal

A

young children
Hand-schuler christrian triad
many spontaneous regress or chemo

192
Q

Hand-schuler christrian triad

A

calvarial bone lesions
post pituitary stalk -> DI
exopthalmus

193
Q

pulmonary

A

adult smokers
regress spontaneously upon cessation of smoking
BRAF

Heme II (7 decks)

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