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[The effect of lenalidomide on rare blood disorders: Langerhans cell histiocytosis, multicentric Castleman disease, POEMS syndrome, Erdheim-Chester disease and angiomatosis].
[erdheim-chester disease]
Lenalidomide
has
been
licenced
for
the
treatment
of
multiple
myeloma
and
,
in
2012
,
it
is
used
as
a
standard
treatment
of
relapses
of
the
disease
.
Literature
contains
a
number
of
publications
on
the
effects
of
lenalidomide
in
myelodysplastic
syndrome
,
in
malignant
lymphomas
and
chronic
B
lymphocytic
leukaemia
.
The
effects
of
the
drug
in
rare
diseases
,
however
,
have
not
been
investigated
so
far
.
In
this
paper
,
we
summarize
our
experience
with
lenalidomide
in
rare
blood
disorders
.
We
observed
an
excellent
effect
of
lenalidomide
in
multifocal
aggressive
,
repeatedly
relapsing
Langerhans
cell
histiocytosis
where
it
led
to
complete
remission
.
This
patient
was
treated
with
2
-
chlorodeoxyadenosine
and
with
CHOEP
(
cyclophosphamide
,
etoposide
,
doxorubicin
,
vincristine
and
prednisone
)
chemotherapy
and
high
dose
BEAM
chemotherapy
with
autologous
transplantation
of
haematopoietic
tissue
for
an
early
disease
relapse
.
Following
another
early
relapse
,
the
patient
was
treated
with
lenalidomide
(
25
mg
)
.
Treatment
with
lenalidomide
induced
complete
remission
on
PET-
CT
.
The
patient
was
consolidated
during
the
remission
with
a
reduced
intensity
conditioning
regimen
and
allogeneic
transplantation
of
haematopoietic
tissue
.
Following
allogeneic
transplantation
,
the
patient
has
been
in
full
remission
for
10
months
.
We
further
showed
an
excellent
effect
of
lenalidomide
in
multicentric
Castleman
disease
with
generalized
involvement
of
lymphatic
nodes
,
B
symptoms
and
vasculitis
.
The
patient
was
first
treated
R-CHOP
chemotherapy
(
rituximab
,
cyclophosphamide
,
adriamycin
,
vincristine
and
prednisone
)
.
Due
to
a
lack
of
efficacy
,
this
was
changed
to
the
CVD
combination
(
cyclophosphamide
,
thalidomide
,
dexamethazone
)
.
This
treatment
delivered
complete
remission
but
was
complicated
by
thalidomide-associated
neuropathy
.
Due
to
persistent
neuropathy
,
thalidomide
could
not
be
used
to
manage
further
relapse
and
thus
lenalidomide
(
25
mg
,
11
cycles
)
was
used
.
The
patient
has
been
in
complete
PET-
CT
remission
for
7
months
following
this
treatment
.
We
observed
partial
efficacy
in
Erdheim-
Chester
disease
.
We
used
2
-
chlorodeoxyadenosine
as
part
of
initial
treatment
that
delivered
partial
regression
of
brain
infiltrates
only
;
fluorodeoxyglucose
accumulation
in
the
bones
has
not
changed
.
Lenalidomide
25
mg
was
used
as
second
line
treatment
.
This
led
to
complete
regression
of
CNS
infiltrates
on
MRI
but
fluorodeoxyglucose
accumulation
in
bone
lesions
did
not
change
.
Regression
of
clinical
signs
and
regression
of
fibrosis
of
retroperitoneum
was
achieved
with
an
ongoing
treatment
with
anakinra
.
A
patient
with
multiple
angiomatosis
affecting
the
abdominal
cavity
,
mediastinum
and
vertebrae
and
digestive
tract
had
been
stabilized
with
zoledronate
(
4
mg
once
every
2
months
)
and
thalidomide
(
100
-
200
mg
/
den
)
for
several
years
.
However
,
several
years
of
this
treatment
led
to
severe
neuropathy
.
Consequently
,
we
attempted
to
substitute
thalidomide
for
lenalidomide
.
However
,
10
mg
of
lenalidomide
alone
was
not
sufficiently
effective
and
thus
low
dose
of
50
mg
of
thalidomide
was
added
.
Combined
treatment
with
zoledronate
,
lenalidomide
10
mg
/
day
and
thalidomide
50
mg
/
day
stabilized
the
condition
for
9
months
.
Due
to
relapsed
gastrointestinal
bleeding
the
treatment
had
to
be
changed
after
9
months
to
thalidomide
100
mg
/
day
and
Sandostatin
0
.
1
mg
twice
daily
s
.
c
.
A
patient
with
osteosclerotic
myeloma
and
POEMS
syndrome
was
initially
treated
with
CAD
chemotherapy
(
cyclophosphamide
,
adriamycine
and
dexamethazone
)
that
was
followed
by
tandem
high
dose
chemotherapy
(
melphalan
100
mg
/
m
2
)
and
autologous
transplantation
.
Treatment
with
thalidomide
was
given
due
to
insufficient
efficacy
but
was
not
tolerated
.
Lenalidomide
was
administered
as
the
fourth
line
treatment
.
Even
though
literature
describes
remission
of
POEMS
syndrome
following
lenalidomide
,
four
cycles
did
not
lead
to
remission
in
our
patient
.
We
showed
an
effect
of
lenalidomide
in
Langerhans
cell
histiocytosis
and
in
Castleman
disease
.
The
treatment
led
to
regression
of
brain
infiltrates
in
a
patient
with
Erdheim-
Chester
disease
.
A
dose
of
10
mg
of
lenalidomide
daily
in
combination
with
50
mg
of
thalidomide
stabilized
a
course
of
angiomatosis
.
Lenalidomide
did
not
deliver
the
required
treatment
response
in
a
patient
with
POEMS
syndrome
and
multiple
previous
therapies
.
Diseases
Validation
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"high dose"
symptom
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pyomyositis
systemic capillary leak syndrome
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