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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
"immunodeficiency"
symptom
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
cushing syndrome
dracunculiasis
hirschsprung disease
hodgkin lymphoma, classical
homocystinuria without methylmalonic aciduria
kabuki syndrome
legionellosis
malignant atrophic papulosis
oculocutaneous albinism
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
primary hyperoxaluria type 1
pyomyositis
severe combined immunodeficiency
sneddon syndrome
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated