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[Cold agglutinin disease - no response to glucocorticoids and rituximab, what treatment is best for the 3rd line of therapy? Case report and review of the literature].
[waldenström macroglobulinemia]
Acquired
autoimmune
haemolytic
anaemia
is
divided
according
to
the
characteristics
of
immunoglobulin
causing
haemolysis
.
The
most
frequent
are
haemolytic
anaemia
with
thermal
antibodies
.
They
bind
to
erythrocytes
and
initiate
their
destruction
in
the
reticuloendothelial
system
cells
,
leading
to
extravascular
haemolysis
.
Cold
agglutinin
disease
differs
significantly
from
haemolytic
anaemia
with
thermal
antibodies
.
Agglutination
is
caused
by
monoclonal
antibodies
,
in
most
cases
class
IgM
and
very
rarely
class
IgG
.
Under
cold
conditions
they
bind
to
erythrocytes
and
cause
their
agglutination
and
subsequent
disorder
of
blood
circulation
in
body
parts
with
a
lower
temperature
.
Agglutinins
binding
initiate
the
binding
of
the
complement
to
the
erythrocytes
.
Under
warm
conditions
the
binding
becomes
loose
but
the
parts
of
the
complement
,
which
are
already
bound
,
cause
haemolysis
,
which
is
mainly
of
an
intravascular
nature
.
The
loose
haemoglobin
causes
haemoglobinuria
.
Description
of
a
patient
with
the
disease
.
The
1
st
symptoms
of
the
disease
,
i
.
e
.
anaemia
+
circulatory
disorders
in
the
acral
parts
of
the
body
,
disappearing
under
warm
conditions
followed
with
haemoglobinuria
,
led
to
the
dia-gnosis
of
cold
agglutinin
disease
.
The
1
st
line
treatment
,
prednison
,
did
not
show
any
response
.
The
2nd
line
treatment
used
was
rituximab
and
dexametazon
.
Rituximab
was
administered
in
doses
of
500
mg
/
m
2
to
4
times
in
a
row
in
weekly
intervals
.
Dexametazon
was
administered
in
doses
of
40
mg
from
1
st
to
4
th
day
and
from
15
th
to
18
th
day
of
the
cycle
.
This
treatment
,
however
,
did
not
show
any
response
either
.
Therefore
this
article
brings
an
overview
of
all
publications
regarding
the
disease
treatment
with
the
aim
of
choosing
the
most
effective
treatment
options
in
the
case
of
failure
of
the
monotherapy
using
rituximab
.
The
1
st
line
treatment
for
cold
agglutinin
disease
is
rituximab
in
monotherapy
,
usually
administered
once
per
week
at
least
for
4
weeks
.
This
treatment
shows
a
response
in
about
one
half
of
treated
patients
and
the
remission
duration
median
after
rituximab
administration
is
11
months
.
A
combination
of
rituximab
with
fludarabin
was
more
effective
,
though
more
toxic
;
this
combination
,
in
a
clinical
study
,
led
to
75
%
of
patients
responding
to
treatment
,
including
20
%
experiencing
complete
remission
.
The
treatment
response
median
reached
over
66
months
.
In
a
small
study
(
10
patients
)
an
increase
in
the
amount
of
rituximab
administrations
from
4
to
8
led
to
a
treatment
response
in
6
patients
in
whom
administration
of
4
doses
of
rituximab
had
no
response
.
When
treating
Waldenström
macroglobulinemia
,
effectiveness
of
the
following
drugs
and
their
combinations
was
proven
:
rituximab
,
chlorambucil
,
cyclophosphamide
,
fludarabin
,
bortezomib
,
lenalidomid
,
bendamustin
and
alemtuzumab
.
The
same
drugs
and
treatment
procedures
are
used
for
the
treatment
of
the
cold
agglutinin
disease
as
for
Waldenström
macroglobulinemia
.
Successful
treatment
with
vortezomibem
,
combinations
of
rituximab
+
bendamustin
,
rituximab
+
cyclophosphamide
or
rituximab
+
fludarabin
+
cyclophosphamide
,
were
recorded
in
the
form
of
a
description
as
regards
the
cold
agglutinin
disease
treatment
.
An
important
benefit
is
also
shown
through
treatment
with
the
monoclonal
antibody
antiC
5
,
eculizumab
,
which
is
otherwise
used
for
the
treatment
of
paroxysmal
nocturnal
haemoglobinuria
.
Eculizumab
blocks
the
C
5
element
of
the
component
and
thus
stops
haemolysis
in
a
patient
with
cold
agglutinin
disease
.
As
cold
agglutinin
disease
is
very
rare
,
there
are
only
a
few
clinical
studies
and
when
treating
this
rare
disease
we
have
no
other
option
than
to
take
into
account
the
information
contained
in
the
descriptions
of
the
particular
cases
of
cold
agglutinin
disease
and
the
experience
of
Waldenström
macroglobulinemia
disease
treatment
.
The
discussion
seeks
to
solve
the
issue
regarding
what
3rd
line
treatment
option
to
use
in
the
described
patient
.
Diseases
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Diseases presenting
"in most cases class igm and very rarely class igg"
symptom
waldenström macroglobulinemia
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