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Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman.
[lymphangioleiomyomatosis]
Disorders
of
the
pulmonary
lymphatic
system
include
macro-
and
microcystic
lymphatic
malformations
,
primary
or
secondary
lymphangiectasias
,
generalized
lymphatic
anomalies
,
diffuse
pulmonary
lymphangiomatosis
,
and
combinations
of
lymphatic
and
other
tissue
anomalies
,
including
lymphangioleiomyomatosis
(
LAM
)
.
We
report
a
case
of
a
patient
with
a
newly
defined
entity
classified
as
kaposiform
lymphangiomatosis
(
KLA
)
.
This
50
-
year
-old
nonsmoking
Hispanic
woman
presented
with
a
20
-
year
history
of
cough
,
hemoptysis
,
chyloptysis
,
and
pleuritic
chest
pain
.
Laboratory
evaluation
demonstrated
a
low
normal
platelet
count
,
elevated
d-
Dimer
,
low
normal
fibrinogen
,
and
elevated
fibrin
split
products
.
Chest
computerized
tomography
imaging
showed
enlarged
hypodense
lymph
nodes
in
the
mediastinum
and
hila
,
and
peribronchovascular
thickening
,
without
evidence
of
cystic
parenchymal
lesions
.
Magnetic
resonance
imaging
of
the
chest
showed
cystic
mediastinal
lymph
nodes
with
heterogeneously
increased
T
2
and
decreased
T
1
signal
intensity
.
Fiberoptic
bronchoscopy
revealed
hyperemic
mucosa
with
granular
appearance
suggestive
of
a
submucosal
infiltrative
process
.
Pathological
specimens
revealed
dilated
,
malformed
lymphatic
channels
within
the
pleura
,
pulmonary
septa
,
and
bronchovascular
bundles
,
and
foci
of
perilymphatic
and
intralymphatic
spindle
cells
which
reacted
with
the
Prospero
homeobox
protein
1
(
PROX-
1
)
immunostain
.
The
morphology
and
immunohistochemistry
results
were
consistent
with
a
diagnosis
of
KLA
.
This
newly
recognized
clinical-pathological
entity
among
intrathoracic
lymphatic
anomalies
is
distinguished
from
generalized
lymphatic
anomaly
and
diffuse
pulmonary
lymphangiomatosis
in
part
by
characteristic
hematological
abnormalities
and
hemorrhagic
complications
,
including
hemoptysis
,
as
experienced
by
our
patient
.
Diseases
Validation
Diseases presenting
"platelet count"
symptom
adrenomyeloneuropathy
benign recurrent intrahepatic cholestasis
child syndrome
cholangiocarcinoma
esophageal squamous cell carcinoma
familial mediterranean fever
heparin-induced thrombocytopenia
lymphangioleiomyomatosis
sneddon syndrome
waldenström macroglobulinemia
wiskott-aldrich syndrome
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