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EDITORIAL
The
Di
Guglielmo
Syndrome
I
N 1923, under
the designation
of “erythremic
myelosis,”
Di Guglielmo
described
a “new”
form of proliferative
blood
disease
involving
not the white
cells
but the nucleated
red cells.
His initial
conceptions
of an erythroblastic
proliferation
akin to leukemia
were
apparently
derived
from
a case of mixed
white
cell-red
Although
Di
highly
cell disorder
which
Guglielmo
emphasized!
specific
only
some
classification,
of
methods
the
as
condition
Guglielmo
Di
autonomous
indicate
far
and!
of
proliferation
we
use
therefore,
time,
Di
of
only
a few
cells,
not
complexity
meantime,
of
pro-
therapeutic
the
end-result
to
of
re-assess
Guglielmo
syndrome.
the
disorder
as “an
tissue.
Our
own
studies
The
pure
erythroblastic
that
red
acute,
indicated
such
is
call the
“purity”
indicate
of
that
unequivocal
after
cases
six months
myeloblasts
predominant
cells.
( acute
granulocytic
proliferation
the
revealed
erythropoietic
of revision.
rare,
studies
the
It
the
of the
in need
of nucleated
become
by
has
process.4
have
but
a growing
forms.1
In the
disease
its variations,
stressed
Our
publications
etc.,
he extraordinarily
reported.
myeloblastic
later
views
“mixed”
the
leukemic
which,
in all
continually
eventually
his
antibiotics,
acute
must
been
of striking
course
pathologic
entity”
this
concept
is
that
proliferation
thus
usual
is an
described
in 1917 as “erythroleukemia.”2
that
“erythremic
myelosis”
was an
of his original
for so-called
transfusions,
cases
this
entity,
modification
particularly
longation
most
pathologic
he
gradually
At
the
end,
or
myeloblastic
having
or Six years
increase
almost
an
“pure”
)
leukemia
is
present.
It becomes
several
row;
ant
clear
: 1
stages
2)
mixed
that
disease,
if it runs
erytllroblastic
proliferation.
expressed,
liferative
that
the
disorders.
or
degree,
This
Di Guglielmo
Because
the
highly
out
is in keeping
syndrome
entire
bone
variable
full
its
pictures
with
is one
marrow
result
the
of
not
1)atient
but in the same
1)atient
as uveil. Parenthetically,
that our attempts
to classify
diseases
into neat packages
because
the diseases
have
their
own
( to us ) confusing
one
conceives
of the
entire
hone
marrow,
one, two, or three
or that
We
several
cell
used
proliferation
if only
tinue
Guglielmo
it becomes
of tile bone
proliferations
have
use(l
variations
myelosis”
disease,
tile
Di
the
one
may
term
may
to the
myeloblasts
Di
see
syndrome
easier
marrow
be
through
the
other
hopelessly
stages.
first
etc.,
thought,
previously
the several
participates
only
myeloproto greater
from
patient
to
disease
that
at any
one
be proliferating
time
of tile
either
excessively,
mixed!.
to
form,
although
first
manifestation.
and
the
patient
perhaps
the
may
all
“pure”
encompass
from
the
“Erythroleukemia”
192
through
it should
be stated
are so often
imperfect
ways
of behavior.
If
Guglielmo
svndirome
in the collditioll,
ranging
may
be observed
as the
but by dint of transfusions,
passes
as a proliferative
to realize
cells may
“pure”
myeloblastic
are present
as
course,
proliferation
in the bone
margrowth;
and finally,
3 ) preponder-
erythroblastic-myeloblastic
myeloblastic
lesser
the
) preponderant
the
the
term
the
red
is not
“Erythremic
only
phase
be “saved”
is in some
respects
of the
to cona good
From www.bloodjournal.org by guest on July 28, 2017. For personal use only.
193
Ei)ITORIAL
terni,
both
eIlli)raciflg
contributions
Once
one
first stage
of nucleated
the
diagnosis
that
some
plastic
(
cases
of
( 2 ) The
)
bone
marrow
fecal
shows
be
simply
the
) , it becomes
apparent
“aplastic
anemia
with
Di Guglielmo
syndrome.
the
made
in such
cases?
We
type,
in
extraordinary
but
the
hyperHow,
have
but
found
with
erythrohlastic
reticulocytes
erythropoiesis).
cells
of the
certain
marrow
output
in the
but the
indicate
too,
a condition
of
marrow
blood
are
show
many
forms.
However,
and there
is no
the
feces
Cr
red
“heme
the
level
bone
hyperplasia,
the
usually
megaloblastoid
or elevated,
reticulocyte
in the “pure”
to make
the
of
only
bizarre
B12 conto the
response
B12.
vitamin
anemia.#{176} Here,
( 5 ) The
pioneer
large
numbers
means
essential
for
no
occur
fecal
and,
and
red cell
diagnosis
smears
proliferations,
of a myeloblastic,
be
urobilinogen
quite
output
presumably,
maturation
blood
may
high-indica-
cell survival
time
pigment
diversion”
because
arrest
and
poiesis,
with
“diversion”
of heme
pigment
from
developing
red cell to bile pigment
production.
even
cient
may
nornlochromic
an
disease,
hemolysisThis
may
of low
results
of
of
actually
urobilinogen
tive of increased
slightly
reduced.
This
the
honor
( b ) that
and
are by
may
and
examples
polyploid
types
and
of the serum
is normal
of
presence
forms
diagnosis
elevated
( ineffective
The
nucleated
red
pernicious
to
proliferation
syndrome
1)lood
anemia”
reality
hemolytic
The
it fails
macrocytosis.
administration
( 4)
in
cell
red
features
helpful:
anemia
is of the normocytic.
suggesting
forms
with
centration
tile
complex
peripheral
“refractory
definitive
suggesting
slightly
(3
more
in the
“anerythroblastic”
are
is the
hut
features,
dfldl
(a )
that
larger,
cells
( i.e.,
following
1 ) The
indices
concepts
Guglielmo.
realizes
of the
red
marrow”
then,
the
Di
of
its
show
a fair
of
be only
seen
in
is high
of the
in
lack
channels
very
careful
the
of B12.
ineffective
usual
upon
may
as
erythrowithin
the
inspection,
number
of myeloblasts
suffias well as of an erythroblastic,
proliferation.
Recognition
forms,
of the
Di
where
the large
abnormal
marrow
highly
Guglielnlo
number
picture
syndrome
begins
with
of nucleated
red cells
are quite
characteristic.
study
in
of the
acute
the blood
and the
“Quieter”
forms
may
then be recognized,
in which
only a few nucleated
red cells in the blood
are
seen,
but with
a fairly
characteristic
marrow
picture.
If splenectomy
is done
in such cases,
a great
increase
in nucleated
red cells of the blood
usually
takes
place
without
any
apparent
beneficial
effect.
Once
diagnosis
of the
acute
forms
ilas
process
unusually
hone
there
the
been
mastered,
take
may
well
marrow
may be
“preleukemic
slow
development
the
myeloblastosis
years
for
documented
the chronic
forms
its final development,
cases,
that
of an
shows
erythroblastic
an occasional
nucleated
status”
of some
of a proliferative
may
at first
may
up
army
be recognized.
Here,
the
to 10 years
in one of our
colonel.
In such
cases,
the
hyperplasia-the
anemia
red cell or myeloblast
authors.
process
be so slight
This
probably
of the
entire
as to have
is “refractory”;
in the blood,
i.e.,
represents
bone
little
the
if any
rather
in which
marrow
effect
on
the
From www.bloodjournal.org by guest on July 28, 2017. For personal use only.
194
EDITORIAL
patient.
Later,
anemia
becomes
peutic
procedures,
outspoken
and presumably
more
marked.
the
leukemia
our
course
as the
of the several
features
the use of the rather
understanding
because
Eventually,
is one
of progressive
terminal
broad
concept
will
anemia”
of the
the
thera-
usually
It is hoped
Guglielmo
syndrome
term
“refractory
rather
erythropoiesis,
of various
use
deterioration,
manifestation.
of the Di
meaningless
of the
of the ineffective
despite
the
that
reduce
and
with
recognition
still further
will improve
myeloproliferative
syn-
drome.
WILLIAM
DAMESHEK
MIauo
BALDINI
REFERENCES
1.
Di
Gugliehuio,
2.
--:
Ricerche
3.
--
:
Acute
1956
4.
Martin,
or
5.
G. : Eritremie
di
erythremic
(in
press).
W.
J. and
incomplete
Dameshek,
Acute.
Ematologia.
Atti
I. Un
disease,
Bayrd,
variety.
W. : Some
E.
Proc.
D.:
Report
Congr.
caso
di
Ataliano
eritoleueeinia.
VI
Congr.
speculations
five
on
with
cases.
the
Int.,
med.
Intern.
Erythroleukemia,
of
Med.
Folia
Blood
Soc.
1923.
Hematology,
special
9:321,
Roma,
17:1917.
emphasis
Boston,
on
the
acute
1954.
myeloproliferative
syndromes.
Blood
6:372,
1951.
6.
London,
I.
blood
cell
7.
Block,
1953.
M.,
Shemin,
dynamics
in
anemia
and
M.
Jacobson,
and
D.,
West,
normal
R.,
humans
pernicious
anemia.
L.
0.:
Preleukemic
and
and
J. Biol.
Rittenberg,
in
D.:
subjects
Chein.
acute
Heme
synthesis
with
polycythemia
179:463,
1949.
human
leukemia.
J.A.M.A.
and
vera,
red
sickle
152:1018,
From www.bloodjournal.org by guest on July 28, 2017. For personal use only.
1958 13: 192-194
Editorial−−he Di Guglielmo Syndrome
WILLIAM DAMESHEK and MARIO BALDINI
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