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MYD88 mutant lymphoplasmacytic lymphoma/Waldenström macroglobulinemia has distinct clinical and pathological features as compared to its mutation negative counterpart.
[waldenström macroglobulinemia]
In
a
first
series
from
India
,
we
report
32
cases
of
lymphoplasmacytic
lymphoma
/
Waldenström
macroglobulinemia
(
LPL
/
WM
)
over
7
years
.
Here
,
we
analyzed
32
patients
with
LPL
/
WM
for
MYD
88
L
265
P
mutation
and
correlated
mutation
staus
with
hematological
and
biochemical
parameters
and
also
with
the
International
Prognostic
Scoring
System
(
ISSWM
)
and
treatment
response
.
Twenty
-
seven
out
of
32
cases
of
LPL
/
WM
(
84
.
3
%
)
harbored
the
MYD
88
L
265
P
mutation
.
MYD
88
wild-
type
WM
was
associated
with
a
lower
number
of
tumor
cells
(
p
<
0
.
01
)
and
older
age
(
p
=
0
.
02
)
and
a
lower
ISSWM
score
at
presentation
(
p
=
0
.
03
)
as
compared
to
mutated
LPL
/
WM
.
On
evaluation
of
response
(
n
=
23
)
,
44
.
4
%
of
patients
with
MYD
88
mutated
LPL
/
WM
had
progressive
disease
,
whereas
no
patient
in
the
MYD
88
unmutated
group
changed
their
baseline
status
.
We
confirm
the
high
frequency
of
MYD
88
mutations
in
LPL
/
WM
.
Although
the
number
of
MYD
88
wild-
type
cases
was
limited
,
our
data
indicate
that
MYD
88
may
represent
an
adverse
prognostic
marker
for
LPL
/
WM
.
Diseases
Validation
Diseases presenting
"tumor cells"
symptom
alpha-thalassemia
carcinoma of the gallbladder
cholangiocarcinoma
cushing syndrome
dedifferentiated liposarcoma
dentin dysplasia
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
junctional epidermolysis bullosa
kindler syndrome
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
severe combined immunodeficiency
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
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