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Hodgkin lymphoma: 2014 update on diagnosis, risk-stratification, and management.
[hodgkin lymphoma, classical]
Hodgkin
lymphoma
(
HL
)
is
an
uncommon
B-
cell
lymphoid
malignancy
affecting
9
,
200
new
patients
annually
and
representing
approximately
11
.
5
%
of
all
lymphomas
in
the
United
States
.
HL
is
composed
of
two
distinct
disease
entities
;
the
more
commonly
diagnosed
classical
HL
and
the
rare
nodular
lymphocyte-predominant
HL
.
Nodular
sclerosis
,
mixed
cellularity
,
lymphocyte
depletion
,
and
lymphocyte-rich
HL
are
subgroups
under
the
designation
of
classical
HL
.
An
accurate
assessment
of
the
stage
of
disease
in
patients
with
HL
is
critical
for
the
selection
of
the
appropriate
therapy
.
Prognostic
models
that
identify
patients
at
low
or
high
risk
for
recurrence
,
as
well
as
the
response
to
therapy
as
determined
by
positron
emission
tomography
scan
,
are
used
to
optimize
therapy
.
Initial
therapy
for
HL
patients
is
based
on
the
histology
of
the
disease
,
the
anatomical
stage
,
and
the
presence
of
poor
prognostic
features
.
Patients
with
early
stage
disease
are
treated
with
combined
modality
strategies
using
abbreviated
courses
of
combination
chemotherapy
followed
by
involved-field
radiation
therapy
,
while
those
with
advanced
stage
disease
receive
a
longer
course
of
chemotherapy
often
without
radiation
therapy
.
Management
of
relapsed
/
refractory
disease
:
High
-dose
chemotherapy
(
HDCT
)
followed
by
an
autologous
stem
cell
transplant
(
ASCT
)
is
the
standard
of
care
for
most
patients
who
relapse
following
initial
therapy
.
For
patients
who
fail
HDCT
with
ASCT
,
brentuximab
vedotin
,
palliative
chemotherapy
,
nonmyeloablative
allogeneic
transplant
,
or
participation
in
a
clinical
trial
should
be
considered
.
Diseases
Validation
Diseases presenting
"high risk"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
adrenal incidentaloma
alpha-thalassemia
aniridia
canavan disease
congenital diaphragmatic hernia
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
hodgkin lymphoma, classical
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
krabbe disease
legionellosis
liposarcoma
locked-in syndrome
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
phenylketonuria
primary hyperoxaluria type 1
severe combined immunodeficiency
sneddon syndrome
waldenström macroglobulinemia
werner syndrome
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