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Allogeneic bone marrow transplantation appears to ameliorate IgA nephropathy in a patient with X-linked thrombocytopenia.
[wiskott-aldrich syndrome]
Wiskott-
Aldrich
syndrome
(
WAS
)
is
caused
by
a
mutation
in
the
WAS
gene
,
and
it
is
clinically
characterized
by
the
triad
of
thrombocytopenia
,
eczema
and
immunodeficiency
.
X-
linked
thrombocytopenia
(
XLT
)
,
which
is
a
clinically
mild
form
of
WAS
,
is
also
caused
by
a
WAS
gene
mutation
.
Patients
with
WAS
/
XLT
sometimes
also
have
autoimmune
diseases
such
as
IgA
nephropathy
.
Progression
of
IgA
nephropathy
may
lead
to
chronic
renal
failure
with
a
poor
prognosis
.
Here
,
we
describe
an
XLT
patient
who
also
had
IgA
nephropathy
.
The
patient
underwent
bone
marrow
transplantation
(
BMT
)
because
of
an
associated-
lymphoproliferative
disorder
,
and
clinical
and
histological
improvement
in
his
IgA
nephropathy
was
observed
after
BMT
.
The
amount
of
galactose-
deficient
IgA
in
the
patient
's
serum
markedly
decreased
after
BMT
.
Therefore
,
immunological
reconstitution
might
improve
autoimmune
diseases
in
patients
with
WAS
/
XLT
.
Diseases
Validation
Diseases presenting
"and it is clinically characterized by the triad of thrombocytopenia"
symptom
wiskott-aldrich syndrome
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