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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
"edema"
symptom
acute rheumatic fever
adrenomyeloneuropathy
aniridia
cadasil
canavan disease
classical phenylketonuria
coats disease
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
erythropoietic protoporphyria
esophageal carcinoma
fabry disease
familial mediterranean fever
focal myositis
harlequin ichthyosis
homocystinuria without methylmalonic aciduria
inclusion body myositis
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
pleomorphic liposarcoma
systemic capillary leak syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
This symptom has already been validated
