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Transplantation outcomes for severe combined immunodeficiency, 2000-2009.
[severe combined immunodeficiency]
The
Primary
Immune
Deficiency
Treatment
Consortium
was
formed
to
analyze
the
results
of
hematopoietic-cell
transplantation
in
children
with
severe
combined
immunodeficiency
(
SCID
)
and
other
primary
immunodeficiencies
.
Factors
associated
with
a
good
transplantation
outcome
need
to
be
identified
in
order
to
design
safer
and
more
effective
curative
therapy
,
particularly
for
children
with
SCID
diagnosed
at
birth
.
We
collected
data
retrospectively
from
240
infants
with
SCID
who
had
received
transplants
at
25
centers
during
a
10
-
year
period
(
2000
through
2009
)
.
Survival
at
5
years
,
freedom
from
immunoglobulin
substitution
,
and
CD
3
+
T
-
cell
and
IgA
recovery
were
more
likely
among
recipients
of
grafts
from
matched
sibling
donors
than
among
recipients
of
grafts
from
alternative
donors
.
However
,
the
survival
rate
was
high
regardless
of
donor
type
among
infants
who
received
transplants
at
3
.
5
months
of
age
or
younger
(
94
%
)
and
among
older
infants
without
prior
infection
(
90
%
)
or
with
infection
that
had
resolved
(
82
%
)
.
Among
actively
infected
infants
without
a
matched
sibling
donor
,
survival
was
best
among
recipients
of
haploidentical
T
-
cell-depleted
transplants
in
the
absence
of
any
pretransplantation
conditioning
.
Among
survivors
,
reduced-intensity
or
myeloablative
pretransplantation
conditioning
was
associated
with
an
increased
likelihood
of
a
CD
3
+
T
-
cell
count
of
more
than
1000
per
cubic
millimeter
,
freedom
from
immunoglobulin
substitution
,
and
IgA
recovery
but
did
not
significantly
affect
CD
4
+
T
-
cell
recovery
or
recovery
of
phytohemagglutinin-induced
T
-
cell
proliferation
.
The
genetic
subtype
of
SCID
affected
the
quality
of
CD
3
+
T
-
cell
recovery
but
not
survival
.
Transplants
from
donors
other
than
matched
siblings
were
associated
with
excellent
survival
among
infants
with
SCID
identified
before
the
onset
of
infection
.
All
available
graft
sources
are
expected
to
lead
to
excellent
survival
among
asymptomatic
infants
.
(
Funded
by
the
National
Institute
of
Allergy
and
Infectious
Diseases
and
others
.
)
.
Diseases
Validation
Diseases presenting
"excellent survival among infants with scid identified before the onset of infection"
symptom
severe combined immunodeficiency
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