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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
"lymphoma"
symptom
adrenal incidentaloma
alpha-thalassemia
carcinoma of the gallbladder
cushing syndrome
dedifferentiated liposarcoma
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
familial hypocalciuric hypercalcemia
focal myositis
hirschsprung disease
hodgkin lymphoma, classical
kabuki syndrome
liposarcoma
locked-in syndrome
monosomy 21
oculocutaneous albinism
primary effusion lymphoma
severe combined immunodeficiency
systemic capillary leak syndrome
waldenström macroglobulinemia
wiskott-aldrich syndrome
This symptom has already been validated