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Spontaneous heparin-induced thrombocytopenia syndrome: 2 new cases and a proposal for defining this disorder.
[heparin-induced thrombocytopenia]
The
existence
of
spontaneous
heparin-induced
thrombocytopenia
(
HIT
)
syndrome
(
or
autoimmune
HIT
)
,
defined
as
a
transient
prothrombotic
thrombocytopenic
disorder
without
proximate
heparin
exposure
serologically
indistinguishable
from
HIT
,
is
controversial
.
We
describe
2
new
cases
presenting
with
thrombotic
stroke
/
thrombocytopenia
:
one
following
shoulder
hemi-arthroplasty
(
performed
without
heparin
)
and
the
other
presenting
to
the
emergency
room
without
prior
hospitalization
,
heparin
exposure
,
or
preceding
infection
.
Both
patients
tested
strongly
positive
for
anti-
platelet
factor
4
(
PF
4
)
/
heparin
immunoglobulin
(
Ig
)
G
in
2
different
immunoassays
and
in
the
platelet
serotonin-release
assay
.
Crucially
,
both
patients
'
sera
also
caused
strong
(
>
80
%
)
serotonin
release
in
the
absence
of
heparin
,
a
serologic
feature
characteristic
of
delayed
-onset
HIT
(
ie
,
where
heparin
use
precedes
HIT
but
is
not
required
for
subsequent
development
or
worsening
of
thrombocytopenia
)
.
We
propose
that
a
rigorous
definition
of
spontaneous
HIT
syndrome
should
include
otherwise
unexplained
thrombocytopenia
/
thrombosis
without
proximate
heparin
exposure
and
with
anti-
PF
4
/
heparin
IgG
antibodies
that
cause
strong
in
vitro
platelet
activation
even
in
the
absence
of
heparin
.
Diseases
Validation
Diseases presenting
"transient prothrombotic thrombocytopenic disorder"
symptom
heparin-induced thrombocytopenia
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