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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
"chest pain"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
dedifferentiated liposarcoma
fabry disease
familial mediterranean fever
hydrocephalus with stenosis of the aqueduct of sylvius
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
pyomyositis
thoracic outlet syndrome
This symptom has already been validated