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Hemophagocyctic lymphohistiocytosis developed in a Japanese boy with Chédiak-Higashi syndrome.
[oculocutaneous albinism]
Chédiak-
Higashi
syndrome
(
CHS
)
is
one
of
the
primary
immunodeficiency
syndromes
accompanied
by
oculocutaneous
albinism
.
It
is
characterized
by
existence
of
giant
granule
of
neutrophils
,
and
development
of
symptoms
of
hemophagocytic
lymphohistiocytosis
.
CHS
is
a
rare
disorder
and
recognition
of
the
disease
is
indispensable
for
its
diagnosis
.
In
our
case
,
a
four
-
month
-old
boy
,
virus-associated
hemophagocytic
syndrome
(
VAHS
)
was
suspected
from
generation
of
fever
,
hepatosplenomegaly
,
and
existence
of
atypical
lymphocytes
on
admission
.
However
,
elevation
of
serum
AST
,
LDH
and
ferritin
were
quite
slight
as
VAHS
,
and
rapid
exacerbation
of
the
findings
was
not
seen
.
Associated
virus
was
undetected
.
He
was
finally
diagnosed
as
CHS
developing
hemophagocyctic
lymphohistiocytosis
based
on
the
existence
of
a
giant
granule
of
neutrophils
in
the
peripheral
blood
smear
and
oculocutaneous
albisum
and
laboratory
findings
.
Clinical
outcome
was
successful
after
receiving
HLA-matched
unrelated
bone
marrow
transplantation
.
Diseases
Validation
Diseases presenting
"blood smear"
symptom
lamellar ichthyosis
oculocutaneous albinism
systemic capillary leak syndrome
typhoid
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