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Utility of LRF/Pokemon and NOTCH1 protein expression in the distinction between nodular lymphocyte-predominant Hodgkin lymphoma and classical Hodgkin lymphoma.
[hodgkin lymphoma, classical]
Classical
Hodgkin
lymphoma
(
CHL
)
and
nodular
lymphocyte-predominant
Hodgkin
lymphoma
(
NLPHL
)
are
considered
separate
entities
with
different
prognosis
and
treatment
.
However
,
morphologic
features
can
be
similar
and
immunohistochemical
studies
are
essential
in
the
distinction
;
thus
,
determination
of
additional
biomarkers
is
of
utmost
importance
.
LRF
/
Pokemon
is
a
proto-oncogene
,
an
interacting
partner
co
-expressed
with
BCL
6
in
germinal
centers
and
highly
expressed
in
diffuse
large
B-
cell
lymphoma
and
follicular
lymphoma
.
Conversely
,
loss
of
the
LRF
gene
in
mouse
hematopoietic
stem
cells
results
in
complete
block
of
early
B
cell
development
with
concomitant
Notch
de
-repression
,
indicating
its
critical
role
in
B
versus
T
cell
fate
decision
at
the
hematopoietic
stem
cell
stage
.
For
the
first
time
,
we
show
that
LRF
/
Pokemon
is
predominantly
expressed
in
NLPHL
cases
as
is
BCL
6
with
low
to
absent
NOTCH
1
protein
expression
;
while
Hodgkin
Reed
-
Sternberg
(
HRS
)
cells
in
CHL
show
low
to
absent
BCL
6
and
LRF
/
Pokemon
expression
with
higher
NOTCH
1
expression
.
We
illustrate
a
potential
functional
interaction
between
LRF
and
BCL
6
in
NLPHL
pathogenesis
,
and
differential
expression
of
LRF
/
Pokemon
and
NOTCH
1
proteins
in
CHL
thus
showing
differential
expression
,
making
for
an
additional
diagnostic
marker
and
therapeutic
target
.
Diseases
Validation
Diseases presenting
"first time"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
gm1 gangliosidosis
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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