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Diverse clinicopathologic features in human herpesvirus 8-associated lymphomas lead to diagnostic problems.
[primary effusion lymphoma]
Human
herpesvirus
8
(
HHV
8
)
-
associated
lymphomas
are
uncommon
,
mainly
affect
men
infected
with
the
human
immunodeficiency
virus
(
HIV
)
,
and
usually
have
a
poor
prognosis
.
We
sought
to
characterize
the
HHV
8
+
lymphomas
seen
at
our
institution
since
the
mid-
1990
s
.
We
identified
15
patients
with
HHV
8
-
associated
lymphomas
and
evaluated
their
clinical
and
pathologic
features
.
Diagnoses
included
primary
effusion
lymphoma
(
PEL
)
(
n
=
2
)
,
extracavitary
PEL
(
n
=
8
)
,
intravascular
large
B-
cell
lymphoma
(
n
=
1
)
,
HHV
8
+
plasmablastic
microlymphoma
(
n
=
3
)
,
and
germinotropic
lymphoproliferative
disorder
(
GLD
)
(
n
=
1
)
.
The
case
of
GLD
progressed
to
a
high
-grade
HHV
8
+
Epstein-
Barr
virus-
positive
lymphoma
,
an
evolution
that
has
not
been
previously
reported
.
Four
patients
were
HIV-
(
three
from
an
HHV
8
-
endemic
area
)
.
Potentially
misleading
pathologic
features
in
our
series
of
extracavitary
PEL
included
classic
Hodgkin
lymphoma
-like
features
,
lymph
node
sinus
involvement
,
and
T
-
cell
antigen
expression
.
H
HV
8
-
associated
lymphomas
can
be
clinically
and
pathologically
heterogeneous
,
with
features
that
may
lead
to
misdiagnosis
as
other
types
of
lymphoma
.
Diseases
Validation
Diseases presenting
"poor prognosis"
symptom
adrenal incidentaloma
alexander disease
alpha-thalassemia
carcinoma of the gallbladder
cholangiocarcinoma
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
junctional epidermolysis bullosa
kindler syndrome
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
papillon-lefèvre syndrome
pleomorphic liposarcoma
primary effusion lymphoma
pyomyositis
sneddon syndrome
systemic capillary leak syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
zellweger syndrome
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