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Neurofibromatosis and childhood leukaemia/lymphoma: a population-based UKCCSG study.
[monosomy 21]
There
is
a
well-known
raised
risk
of
leukaemia
in
children
with
neurofibromatosis
type
1
(
NF-
1
)
.
We
carried
out
the
first
detailed
population-based
study
of
leukaemia
and
non-
Hodgkin
lymphoma
(
NHL
)
associated
with
NF-
1
in
order
to
estimate
the
risk
and
elucidate
the
relationship
between
these
conditions
.
Over
the
17
year
study
period
there
were
five
cases
of
chronic
myelomonocytic
leukaemia
(
CMML
)
in
patients
with
NF-
1
(
relative
risk
221
;
95
%
CI
71
-
514
)
,
12
cases
of
acute
lymphoblastic
leukaemia
(
ALL
)
(
relative
risk
5
.
4
;
95
%
CI
2
.
8
-
9
.
4
)
and
five
cases
of
NHL
(
relative
risk
10
.
0
;
95
%
CI
3
.
3
-
23
.
4
)
.
Marrow
cytogenetics
could
be
reviewed
for
seven
patients
.
Specific
abnormalities
found
were
monosomy
21
in
a
child
with
CMML
and
7
p
+
,
17
p
-
in
a
child
with
ALL
.
No
abnormalities
were
reported
of
17
q
,
which
includes
the
NF
1
gene
.
CMML
occurred
predominantly
in
boys
,
who
also
had
a
family
history
of
NF-
1
.
ALL
and
NHL
were
more
often
found
in
children
with
no
previous
family
history
.
