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Immunogammopathy maculopathy associated with waldenström macroglobulinemia is refractory to conventional interventions for macular edema.
[waldenström macroglobulinemia]
To
describe
immunogammopathy
maculopathy
in
the
setting
of
Waldenström
macroglobulinemia
and
its
lack
of
response
to
ocular
treatment
.
Case
report
of
a
65
-
year
-old
male
patient
who
presented
with
a
4
-
month
history
of
progressive
vision
loss
in
the
left
eye
.
Initial
ophthalmic
examination
showed
bilateral
central
retinal
vein
occlusion
with
macular
edema
,
including
serous
macular
detachment
in
the
left
eye
.
Serum
protein
electrophoresis
revealed
elevated
IgM
levels
,
leading
to
a
diagnosis
of
Waldenström
macroglobulinemia
.
The
patient
underwent
plasmapheresis
and
systemic
chemotherapy
with
improvement
of
hyperviscosity
retinopathy
.
However
,
the
macular
detachment
and
edema
in
the
left
eye
persisted
despite
repeated
intravitreal
injections
of
bevacizumab
.
Panretinal
photocoagulation
and
intravitreal
corticosteroid
treatment
were
also
unsuccessful
in
reversing
the
maculopathy
.
A
diagnosis
of
immunogammopathy
maculopathy
was
made
,
and
partial
improvement
was
seen
over
time
after
the
treatment
of
Waldenström
macroglobulinemia
.
In
the
setting
of
systemic
immunogammopathy
,
conventional
treatments
for
macular
edema
,
including
intravitreal
anti-
vascular
endothelial
growth
factor
agents
and
corticosteroids
,
may
be
ineffective
.
Ocular
involvement
responds
to
systemic
chemotherapy
and
plasmapheresis
directed
against
the
underlying
hematologic
malignancy
,
although
this
response
may
be
slow
and
incomplete
.
Diseases
Validation
Diseases presenting
"retinopathy"
symptom
alpha-thalassemia
cadasil
cholangiocarcinoma
coats disease
cohen syndrome
congenital toxoplasmosis
cowden syndrome
homocystinuria without methylmalonic aciduria
neonatal adrenoleukodystrophy
oculocutaneous albinism
pyomyositis
sneddon syndrome
von hippel-lindau disease
waldenström macroglobulinemia
This symptom has already been validated