From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
Systemic
Mastocytosis
in a Patient
With
Polycythemia
Vera Treated
With
Radioactive
Phosphorus
By James
W.
Eagan,
Jr.,
C. Lockard
Systemic
mastocytosis
event
in
a
polycythemia
treated
occurred
patient
vera.
The
the
course
over
radioactive
with
ships
between
themia
vera,
as
also
of
noted.
The
to be described
of32P,
with
with
pancytopenia
cell
rare
benign
more
are
establishing
disease
the
in this
diagnosis
setting
are
also
with
the occurrence
of
transformation
has been
vera treated
with ioniz-
vera
x-ray
developed
21 yr.
appeared,
therapy
and
during
Within
a year
6 wk
later
the
in the
form
of
skin
early
adult
last
patient
for
life
dose
died
with
has not
exposure.
urticaria
been
In
pigmentosa
of polycythemia
vera.57
mastocytosis
to polycythemia
vera
and
Difficulties
encountered
in establishing
nosis
also
are
(32P).”3
been
of the
type of mast cell disease
vera or after
radiation
mastocytosis
not
to the
preceded
the appearance
Possible
relationships
of
with radiation
are discussed.
cell disease
elec-
illustrated.
of radioactive
phosphorus
in these
patients
has
32P for
neoplasm.
This
with polycythemia
has
of mast
radiation,
and
findings
in
cell
superficial
Polycythemia
a disseminated
mast
reported
in association
instances,
ionizing
described.
received
controlled
progressive
Difficulties
of mast
polycy-
with
Histopathologic
microscopic
instances
in the form
of other
neoplasms
as an adolescent.
successfully
treatment
discussed.
VERA
occasionally
terminates
The frequency
of this lethal
in patients
with polycythemia
in most
incidence
was
sis and
Miller,
mastocyto-
ing radiation,
An increased
psoriasis
C. Eggleston
tron
relation-
between
OLYCYTHEMIA
acute
leukemia.”2
reported
to be increased
and
yr
21
Stanley
Joseph
are
been
and
P
patient
a fatal
had
Possible
mastocytosis
and
L. Baughman,
and
long-standing
patient
phosphorus.
Kenneth
Conley,
treatment
the diag-
described.
CASE REPORT
The
patient
was
he had
developed
obtained.
The
At age
27,
flushed
he
noted
From
onset
January
in part
Hospital.
7. 1976;
hr
NIH
and
Baltimore,
accepted
Grant
after
was
Hopkins
treatments
weight
the
onset
of
observed
to
have
psoriatic
skin
Medicine.
The
with
loss,
these
at
bleeding
gums,
Hopkins
he
was
appearance,
and
15 yr.
was
radiation
symptoms
changes.
Johns
local
age
biopsy
headache,
a plethoric
At
Hospital.
although
a spleen
Hospital.
17.
and
admitted
to
suffusion
of
palpable
and
not
age
the
2 cm
Depart-
Md.
November
5 T0IA
seven
malaise,
mild
of Pathology
Sinai
Supported
he
engorgement.
the Departments
Submitted
of
I yr
where
venous
of Medicine.
Digestive
insidious
Baltimore,
six or
following
Approximately
in
conjunctivae,
M05260
20.
1976.
from
the Institute
of A rthritis,
Metabolism,
and
Diseases.
A ddress
Hopkins
1977
Blood,
the
to the Iohns
felt clinically to be typical of psoriasis,
lesions improved
Hospital
,nent
at the time of his admission
yr
skin changes
appearance.
Sinai
the
49
Vol.
for
reprints
requests:
Hospital.
Baltimore.
by Grune
& Stratton.
49,
No.
4 (April),
James
Md.
W. Eagan.
Jr.,
M.D..
Department
of Pathology.
The
Johns
21205.
Inc.
1977
563
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564
EAGAN
below
the
count
of
left
costal
an erythrocyte
studies
were
The
32P
margin.
19,600/cu
mm,
rate
performed.
Arterial
was
thought
intravenously.
trolled
In
with
mCi
was
During
the
tocrit
Five
nadir
months
and
continued
blasts
with
of
32P.
a reticulocyte
at rest
vera.
the
his
dose
of
74.5
following
that
treatment.
he
tion
to granulocytic
The
patient
(20
mg/day)
A
leftward
granulocytes
(Fig.
time
1.0”,,.
cell
smear,
Blood
volume
6.65
symptoms
1). The
the
mCi
were
final
patient
progressive
Over
of
con-
dose
of
appeared
next
marrow
count
Sinai
granulopoiesis
to
7.0
be
an
marrow
pancytopenia
and
the
early
in
mg/day)
a stable
a hemamm.
fever
appearance
stage
aplasia
ISO
with
of 57,000/cu
Hospital
with
and
suggested
(oxymethalone,
7 mo,
pancytopenia
and platelet
admitted
to
32P-induced
steroids
the
mm,
was
blood
from
anabolic
begun.
in
peripheral
Recovery
transfused,
were
shift
in the
leukemia.
was
blood
peripheral
he received
and
mCi
developed
18#{176}-,,,
white
blood
cell count
of I 100/cu
following
the last dose
of 32P, the patient
immature
of
venesection
at which
a white
the
96”,,.
erythrocytosis
admission,
79”,,,
on
count
was
After
patient’s
a total
to
of
as estimated
AL.
of 50”,,.
pancytopenia.
and
and
saturation
yr.
with
prior
a hematocrit
elevated
polycythemia
21
a hematocrit
4 mo
of
oxygen
have
14 mo
included
markedly
of 0 mm/hr.
ensuing
I I injections
administered
health,
robust
to
the
data
count
sedimentation
not
patient
Laboratory
a platelet
El
of
of transforma-
was
also
and
later
considered.
prednisone
transfusion
requirement
persisted.
Approximately
fever,
13 mo
cough,
that
time.
and
marked
tinued.
nausea,
During
the
poikilocytosis
of
I mo
hospitalization,
evaluation.
The
tenderness,
Chiari
The
of
biopsy
less
spleen
or
increase
the
cells,
marrow
below
noted.
and
hepatitis
numerous
the
immediate
and
Johns
and
immaturity
of
were
occurred.
Hopkins
deeply
icteric,
costal
tests
margin.
to
rule
the
noted.
Androgen
and
was
platelet
performed
increased
was
the
Budd-
biopsy
platelet
showed
fibers.
On
splenectomy
complicated
the
and
count
by
of
bone
infection,
reticulin
fur-
span
lesions,
persisted.
with
transfusions,
period
skin
marrow
bleeding
for
a liver
tumor,
Bone
gastrointestinal
Hospital
with
No
out
unrevealing.
megakaryocytes,
postoperative
and
WBC),
basophils
bleeding
the
and
left
were
laparotomy
splenectomy
The
to
ill
Further
and
num-
myelocytes,
(27/100
showed
and
discon-
empirically.
transferred
acutely
was
31”,,
at
effusion,
Increased
cells
cells
with
noted
pleural
thereafter.
blasts,
Sinai
was
Oxymethalone
again
of mast
begun
to
left
blood
gastrointestinal
was
was
transient
(5”,,
red
admitted
Splenomegaly
tests.
examination
and
again
rapidly
smear
Clumps
were
exploratory
function
tapered
of nucleated
worsened
Despite
mm.
peripheral
13 cm
obstruction,
an
liver.
in the
appeared
palpable
thrombocytopenia
20,000/cu
and
patient
he
in immature
liver
mg/day
Bone
adenopathy
hospitalization,
a
therapy
the
now
biliary
patient’s
of
than
was
developed
patient
deteriorated,
admission,
palpable
syndrome,
a marked
day
On
the
80
Antituberculous
was
urine.
erythropoiesis.
condition
resumed.
patient
dark
appearance
cells.
diminished
general
was
17 cm.
red
and
the
of
appeared
the
of 32P,
appearance
abnormal
to
with
mature
series
patient’s
After
with
increased
associated
granulocytic
dose
the
course.
granulocytes
30#{176},,
juveniles).
ther
final
and
hospital
was
bersofimmature
therapy
the
hepatosplenomegaly
Prednisone
The
after
vomiting,
ninth
wedge
remained
moderate
upper
70
60
50
U
40
4
30
20
FIg.
0
95?
53
55
57
59
6?
63
YEARS
65
67
69
7?
73
1.
Doses
and
times
of administration
the effect on the hematocrlt
development
of pancytopenia.
approximate
of P
prior
and
to the
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SYSTEMIC
MASTOCYTOSIS
Fig. 2.
Diffuse
and eosin. x 400.
565
infiltrate
gastrointestinal
bleeding
came
and
obtunded
platelets,
and
patient
sixth
and
in spleen
fluid
deteriorate.
with
infiltrates.
fever.
to
postoperative
cells
pulmonary
developed
parenteral
continued
the
on
of mast
On
hypotension.
in addition
and
dense
the
and
fifth
postoperative
was
Antibiotics
a massive
upper
Hematoxylin
day
He
steroids.
following
background.
tachycardia.
to high-dose
died
fibrous
the
treated
patient
with
were
beblood,
continued.
gastrointestinal
The
hemorrhage
day.
PATHOLOGY
Surgical
The
Specimens
spleen
On
weighed
fresh
numerous
histologic
efiaced
h
fibrous
of
section
rnarginatcd
cells
along
multinucleate
varied
from
plasm
blue
and
the
many
of
tration.
nificant
or
were
cells
granulated
figures
Alcian
among
architectural
of splenic
for
contained
only
cells
present
cells,
tissue
from
the
electron
microscopy.
a small
number
(Fig.
4).
the
Many
outer
in
appearance
edge
with
(Fig.
2). The
bands
which
the
obtained
marrow
With
of material
originally
from
the
granules.
bounded
was
and
by
this
done
fixed
light
toluidine
noted
as mast
cell
nodes.
without
during
biopsy
and
the cytoplasm
granular
cyto-
reagent.
process
lymph
often
hinucleate
metachromasia
biopsy
were
the
of cells,
a densely
Schilr
identifying
and
abdominal
expected
was
throughout
chromatin.
the majority
exhibited
in the
architecture
Occasional
acid
electron-dense
granules
fibrous
granular
marrow
normal
present
prominent.
In
cells
present
As
of
3).
the
stippled
periodic
(Fig.
biopsy
not
finely
a light
A hone
a finding
pale
was
surrounding
not
rare.
and
distortion.
mast
formal,n),
with
were
and
background
in the liver
was
,nfiltrate
the
oval
were
Occasional
blue
surface
aqueous
Nucleoli
Mitotic
eosinophilic.
cut
cellular
or
metachromatically.
numerous
were
15’,,
nests
membrane.
cells and
were
noted
obtained
in
the
seen.
dlfuse
round
with
firm:
were
A
discrete
nuclear
positively
fragments
formalin
fixed
bands.
generally
stained
the remaining
Similar
cells
Small
most
stained
showed
material
were seen.
to lightl
granules
ver
nodules
forming
were
the
cells
clear
fibrosis
iiation
(all
as
tumor
fibrous
in areas
the
g and
No
interlacing
tissue,
nuclei
1710
infarcts.
7 mo
in
hospitalbefore.
I 5”,, aqueous
microscopic
only
in
infilsig-
a
a well-defined
findings,
few
heavily
limiting
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566
EAGAN
Fig.
3.
Splenic
with
granular
mast
cell
El
infiltrate
AL.
as
toluidine
blue stain.
cytoplasm
which
Densely
stained
metachromatically
is noted
in some
cells (closed
arrow),
with
more sparse
granularity
of remaining
cells
(open
seen
membrane.
The
intragranular
Occasional
sections
matrices
lamellar
of the granules
structures
megakaryocytes
of spleen,
liver,
and
and
were,
were
in general,
Toluidine
uniformly
fine
blue.
x 1500.
to coarsely
granular.
Rare
seen.8
aggregates
lymph
arrow).
nodes
of
nucleated
red
blood
cells
were
identified
in
the
examined.
A utopsv
At
autopsy
lymph
large
nodes,
no increase
process,
numbers
and
bone
in the
with
of mast
cells
were
No
skin
lesions
marrow.
number
of
scattered
mast
cells.
collections
The
of
again
noted
were
kidneys
mast
cells
in the
seen,
were
in
the
liver,
and
the
thoracic
random
only
cortices.
and
sections
other
abdominal
of skin
organs
showed
involved
predominantly
in
with
associated
by
the
perivascular
locations.
Other
major
pleuritis
and
respectively,
The
stomach
The
liver
poiesis.
findings
included
bilateral
pleural
the
latter
and
intestine
weighed
multiple
a
severe
effusion.
containing
a
contained
2780
areas
g.
In
acute
Two
grossly
the
necrosis
ulcers
arterial
amount
to
of centrilobular
duodenal
eroded
a large
addition
bronchopneumonia,
penetrating
of fresh
mast
were
cell
measuring
branch
and
altered
infiltrate
present,
in
its
fibrinous
2 and
bed,
were
1.3 cm,
present.
blood.
and
extramedullary
consistent
with
the
hematoterminal
hypo-
tension.
The
cells.
bone
The
decalcified
nificant
ciated
marrow
was
metachromatic
histologic
chronic
with
Permission
difTusely
sections
pulmonary-
secondary
to examine
hypercellular.
staining
seen
obtained
disease,
polycythemia
the
in
head
from
or
was
and
and
marrow
other
approximately
touch
adjacent
bone.
No
neoplastic.
renal,
or
noted.9
neck
30”,,
preparations
organs
was
not
granted.
40”,,
of the
was
greatly
anatomic
cardiac
cells
were
evidence
lesions
mast
attenuated
usually
in
of
sigasso-
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SYSTEMIC
Fig.
filtrate.
MASIOCYTOSIS
4.
567
Electron
micrograph
x 4100,
enlarged
from
demonstrating
x 2500.
a well-granulated
mast
cell
in splenic
tumor
in-
DISCUSSION
Erythroid
hyperplasia
is considered
majority
of patients
with polycythemia
will also
exhibit
increased
activity
series,
differentiating
The disease
of the causes
the hallmark
of polycythemia
vera. The
vera
at some
time
during
their
course
of the granulocytic
and
megakaryocytic
polycythemia
has thus
of death
vera
been characterized
in these
patients
from
other
forms
as a “panmyelosis,”2
is transformation
The relationship
of this transformation
in the form of radioactive
phosphorus,
Lawrence
et al.’2 have
postulated
of treatment,
the disease
concluded
They
expected
have
because
of the
citing
experience
has averred
that acute
process.
Modan
and
that
leukemic
transformation
noted,
when
however,
compared
that
to
the
that
into
and,
acute
in fact, one
leukemia.”
to treatment
with
radiation,
primarily
has been a subject
of much
debate.
that
the occurrence
of leukemia
or
“leukemia-like
state”
is the result
of a natural
progression
process in polycythemia
vera.
They
have
suggested
that
tion has become
more
prevalent
tients
treated
with 32P. Israels,’3
of erythrocytosis.2”#{176}
of the
leukemic
increased
survival
with nonradioactive
a
basic
disease
transformatime
in pamethods
leukemia
is not a natural
complication
of
in a large
multicenter
study,
have
is chiefly
due
to radiation
therapy.
frequency
seen
of leukemia
in patients
without
has
been
higher
polycythemia
than
vera
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
568
EAGAN
who
have
had
polycythemia
ofleukemia.
similar
amounts
vera may
The patient
developing
leukemia
disorder
and
Another
on the
larger
total
common
development
treatment
with
207 patients
proximate
metaplasia”
of both
death
and
is again
not
with polycythemia
vera
fourfold
increase
in the
in patients
treated
with
pared
Wasserman
those
not
an
in his cases
in some
have
so
of long
led him
treated.
autochthonous
degree.2
part
duration
The
these
appearance
to speculate
than
The
tissue
mast
cell
preexisting
Marshall7
in their
clear.
have
system.
a stimulus
mast cells
Further
is found
Sagher
the
this
is the
to
in a series
of
an increase
in the dewith
radiation
com-
myeloid
that
been
causing
present
polycythemia
with proliferation
tissue
elements
of the
our
and
reports
to osteosclerosis,
not only
as well.2
primitive
classified
among
of mastocytosis
to
and
invariably
stimulus
a derivative
not,
metaplasia
vera,’5
in addition
growth
the
knowledge,
of
mesenchy-
reticuloendoin patients
with
been
recorded,
been
reported.57
In each instance,
cutaneous
mastocytosis
(urticaria
of polycythemia
vera.
Brink
and
concurrence
of the
of the reticuloendothelial
Gross6
have considered
to the “multipotential
and later of the erythroid
noted
treated
almost
changes,
initial
has
Silverstein,’4
polycythemia
considered
view
of
for
vera
considers
of
changes
the
vera
report
relationship
Diamond
and
survival
relationship
isolated
cases ofthe
opposite
sequence
have
the patient
has presented
with long-standing
pigmentosa),
with the subsequent
appearance
of the close
average
The
cell tumors
have
been
Although
the development
polycythemia
with
polycythemia
have
not
patients
ofthese
that
is also
patients
with
vera is directed
at the primitive
mesenchymal
cell,
hematologic
but also of nonhematologic
connective
mal cell, and mast
thelial
neoplasms.’6
that
from
the Mayo
Clinic,
has noted
an apincidence
of “postpolycythemia
myeloid
32P, compared
to those
treated
by other
however,
in their
to
felt
myelofIbrosis.”
entirely
means.
Modan
and Lilienfeld,i
velopment
of myeloid
metaplasia
myelofibrosis
longer
in patients
metaplasia
radiation
and
of 32P.
of
myeloid
exposure
AL.
an increased
susceptibility
to the development
would
have
been considered
at high risk
basis
dose
cause
of
of radiation
also have
presented
El
two
system
their case
reticulum
series.
cells,”
disorders
as evidence
and the hematopoietic
as evidence
suggesting
with
initial
proliferation
of
evidence
of the relationship
of mast cells to the hematopoietic
system
in a review
of 26 fatal
cases
of mastocytosis
from
the literature
by
and
Even-Paz.’7
mastocytosis
(i.e.,
tissue
Twenty-one
of
confirmation
these
of
patients
pathologic
had
proven
increase
of
systemic
mast
cells
in
other
than cutaneous
sites).
Of these
21, at least
S terminated
with
mast
cell
leukemia,
and 3 died with hemocytoblastic,
myeloid,
and monocytic
leukemia,
respectively. An additional
three who did not develop
elevated
white
cell counts
had the appearance
of a significant
percentage
of tissue
mast cells in the peripheral
blood
prior to their demise.
It is also of interest
that the distribution
is primarily
to the organs
involved
by
bone
results
and
marrow,
liver,
spleen,
and lymph
of studies
on the effects
of ionizing
in man.
associated
He
with
remarks
that
a marked
rise
repeated
in the
mast
of therapeutically
cell disease
nodes.’8
Selye’t
radiation
on
exposure
mast
cell count
to
administered
this patient,
in
reviews
the mast
the
cell
ionizing
radiation
in various
tissues.
32P
i.e.,
conflicting
in animals
may
No
be
cases
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
SYSTEMIC
of
MASTOCYTOSIS
cell
sequence
neoplasms
ofevents
mast
The
cytosis
569
associated
in this case
by long-term
Certain
patient
treatment
diagnostic
as mast
metaplasia
with
with
difficulties
cell
with
raises
ionizing
arose
in nature,
radiation
have
the possibility
been
noted,
of induction
radiation.
in identifying
in particular
the
terminal
differentiating
myelofibrosis
however.
of masto-
20
occurring
process
it from
the
in patients
in this
“myeloid
with
polycy-
themia
vera. This patient
presented
many
of the clinical
and laboratory
features
seen in the syndrome,
including
increasing
hepatosplenomegaly,
a leukoerythroblastic blood picture with prominent
poikilocytosis, anemia, extramedullary
hematopoiesis,
sence
of skin
and
increase
lesions
and
in reticulin
some
of the
such as flushing, dermographism,
fibers
more
on
tachycardia
etc.,2’ further
obscured
the diagnosis.
mast cells was noted
in the aspirated
mon
in association
with a variety
splenomegaly
due to various causes.22
bone
specific
marrow
biopsy.
histamine-related
unassociated
The
ab-
symptoms
with
hemorrhage,
Although
an increase
in the number
of
marrow
smear,
this has not been
uncomof’ disorders,
in particular
anemia
and
Because
of sparse
granularity
of the mast
cells in the bone marrow
biopsy, these were initially difficult to recognize among
the immature
marrow
elements.
In hematoxylinand
eosin-stained
sections,
recognition
of’ the occasional
well-granulated
cells led to the establishment
of’
the diagnosis
by use of appropriate
special
stains.
The
sparse
granularity
of the
mast
Fig.
5.
Touch
preparation
from
fresh
resected
spleen
demonstrating
large
number
of
metachromatically
staining
granules
in mast
cells and
background.
Toluidine
blue. x 1500.
cells
may
have
been
due
to
a number
of
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
570
EAGAN
factors, in particular in this case,
granules
are quite
water
soluble
said
to
be
less
granularity
of
In
the
this
cells
the use of routine
aqueous
in certain
species,23 although
case,
of
compared
to touch
preparations
idine blue (Fig.
5). This
patient
final course,
and the use of adrenal
disruption
and
In summary,
treated
neoplasm.
with
this
been
has
may
degranulation
this patient
the
in both
presented
first
such
died
was
case,
the
possibility
of another
tive mesenchyme,
long-term
treatment
was
fixed
marked
Mast
they
cell
are
decrease
in aqueous
and animals.24
a long history
and
disorder
The
proliferation
man
with
reported
coincidence.
there
spleen
AL.
in
formalin
when
from
the fresh
spleen
also stained
with
tolualso received
prednisone
during
much
of his
corticoids
has been associated
with mast cell
radioactive
phosphorus
Diagnosis
of the latter
represent
represent
however,
in sections
fixatives.
in man
El
cell
either
as a result
with ionizing
of polycythemia
concurrence
of the
is raised,
line
vera
with
a disseminated
a source
of difficulty.
of an
two
however,
already
mast
cell
Because
disorders
that
this
may
hyperstimulated
of the basic
nature
radiation,
or both.
primi-
of polycythemia
vera,
of
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PA:
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From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
1977 49: 563-571
Systemic mastocytosis in a patient with polycythemia vera treated with
radioactive phosphorus
JW Jr Eagan, KL Baughman, S Miller, CL Conley and JC Eggleston
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