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Delayed immune recovery following sequential orthotopic liver transplantation and haploidentical stem cell transplantation in erythropoietic protoporphyria.
[erythropoietic protoporphyria]
A
nine
-
yr
-old
boy
with
EPP
suffered
from
severe
skin
burns
and
liver
failure
caused
by
progressive
cholestasis
and
fibrosis
.
OLT
was
performed
without
major
complications
.
Four
months
following
liver
transplantation
he
underwent
parental
haploidentical
HSCT
.
The
myeloablative
conditioning
regimen
was
relatively
well
tolerated
and
hematological
engraftment
was
rapid
(
on
day
10
)
.
Protoporphyrin
concentrations
returned
to
normal
following
HSCT
.
However
,
immune
recovery
was
significantly
delayed
.
Varicella
zoster
virus
reactivation
resulted
in
impaired
vision
,
prolonged
hospitalization
and
eventually
in
multiorgan
failure
and
death
.
Sequential
liver
and
haploidentical
HSCT
proved
feasible
though
a
high
risk
procedure
in
this
EPP
patient
.
The
management
of
post-
IST
after
these
combined
transplantations
remains
a
challenge
and
needs
to
be
further
established
.
Diseases
Validation
Diseases presenting
"skin burns"
symptom
erythropoietic protoporphyria
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