From www.bloodjournal.org by guest on June 16, 2017. For personal use only.
Thrombocytopenic
Purpura
Leukopenia
in
A.
By
T
HR.OMBOCYTOPENIA
bheedimig.
Omi the
heukopeiiia
is
ported.3
there
other
rare
Iii the
‘
which
the
was
present
omily
slight
feature
emilargement
S.,
a 27 year
cutanseous
old
Jewish
hsemisorrhage,
anemia
amid
occasionally
epistaxis,
two
amid
purpura
such
disease
a
by
no
or
re-
is presemited
and
purpimra
amid
is
without
anemia
cases
have
heemi
of Gaucher’s
spleen
leukopenia
is accompanied
thrombocytopenic
of the
female
IZAK
with
a case
was
CASE
C.
G.
thrombocytopemiic
disease.
Only
communicatiomi
clinical
and
Disease
AND
disease
hamid
Gaucher’s
in
main
VRIES
irs Gaucher’s
Anemia
Gaucher’s
associated
fimiding
(OiliIiiOii
in
DE
without
amiemia
or
19,
1953,
iii
which
heukopemiia.
REPORT
clerk
was
gingival
admitted
bleeding
on
and
July
because
of sub-
meno-metrorrhagia.
history.
One brother
died
three
days
after
birth,
deeply
jaundiced.
A sister
aphad jaundice
from birth, remained
physically
and mentally
underdeveloped
and
at the age of six. The mother
died at the age of 53 of cancer
of the breast.
The father,
Family
panemstly
died
60 years
the
of
age,
apparently
is in
Previous
Two
previous
slight
Duninug
the
Present
a year
There
Since
up
seven
(6)
is no
history
of
bleeding
temsdency
in
The
were
,
was
soft
The
spleemi
the
(lie
and
4,37
first
was
palpated
l)all)ated
just.
ferenstial
urine
cent
in
mi
was
6 msiiniumtes,
clotting
test. I)ositive,
pnot.hnonibin
The
Fromsi
Jenusalensu,
Submitted
the
was
the
time
Kahn
I)epartment
hasting
bruised
sternal
hut
4
and
were
nausea,
a few
of
weeks.
times.
The
costal
on
Blood
per
4 pen
clot
Wassermann
legs.
per
blood
were
1955;
accepted
for
band
complete
publications
nodes
red
2 pen
Dif-
cent
eosino-
75 minutes,
cent
imnea,
University
1955.
count
80,000/cu.mm.,
7 per
fibrinogen
Blood
30,
9 mm.
blood
megakaryocytes
The
bleeding
Hadassah
May
Except
9,600/cu.mm.
forms,
after
negative.
tender.
(Westengrenu)
Thrombocytes
B, Rothchild
1055
lymph
not
count
serum
prothnomhin
cent
(normal)
and
tests
The
cent;
was
normal,
with
platelet
formation.
retraction
Division
and
the
nate
cent
monocytes.
bone
marrow
with
diminished
60 per cent,
60 per
firm
on
12 Gm.
white
neutrophils,
cent
was
seen
sedimentation
cent;
nor
ecchymoses
findings.
The abdomens
it was firm arid not tender.
margin;
Hemoglobin
0.9
legs.
and
periods
pallor
Numerous
the
shown
bleeds
abnormal
amid
were
amid had
nose
Menstrual
l)lood loss.
state
with neither
especially
margins
easily,
nsormah.
not reveal
the
normal.
minutes,
of Medicine,
2,
for
as frequent
melena.
Israel.
March
obesity.
by
a number
as well
she
had
body,
did
second.
plasma
prothnomhin
conversion
accelerator
Coonnbs,
fever
had
pressure
costal
amid 5 per
miormal.
The
amid morphology
quet
serum
the
count
22 Per’ cemut lymphocytes
phils,
the
chest
below
polymorph
nnonpisologically
mnal
muunnber
by temporary
accompanied
occurred
patient
1)100(1
over
reticulocvte
coummit : 67 per
the
and
3 cm.
below
26 mm.
nsillion/cim.mni.;
episodes
rio abnormalities
The
amid
grade
hemorrhages
to admission
amid
neck
manifestations
hour
low
accompamsied
by conssiderable
was in good
nutritional
scattered
head,
Lahoraloryfindings.
followed
jatmndice
patient
l)ulse
The
liven
was
liulrlumric
the
were
The
seen
riot emulanged.
similar
childhood
tennl)erature,
pete(’hsiae
a half
mumps
slight
arid
subcutaneous
years
Previous
days
had
15.
early
examination.
jaumidice.
patient
developed
discomfort
and
the age of
illness.
Physical
for
of
at
she
abdominal
amid spontaneous
bleeding.
Three
lasted
cent.
health.
age of 14 the
admission
upper
was
petechiae
ginigival
were
to
course
menuanchse
amid
At the
history.
years
vomiting,
in
good
family.
nortime
t.ourni-
(normal),
380 mg.
per
glucose
Hospital,
and
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1056
THROMBOCYTOPENIC
bihirubin
were
and
tion
4.6 Gm.
X-ray
per
The routine
Ara
cent,
were
and
of the
The
and
peared.
The
the
drip,
test
Gm.
per
5 mg.
daily
on
negative,
thymol
protein
turbidity,
was
were
8.4 Gm.
thymol
floccuha-
pen cent.,
albumin
normal.
purpura
a period
tenth
the
DISEASE
cent.
for
the
GAUCHER’S
tests,
total
electrocardiogram
count
was
serum
thrombocytopenic
thrombocyte
tourniquet
IN
function
The
3.8
of idiopathic
by intravenous
liver
normal.
globulin
chest
diagnosis
ACTH
the
normal.
Takata
PURPURA
day
bleeding
of
was
ten
made.
days.
of treatment
time
The
No
had
5 minutes,
risers
the
patient.
niew
was
given
hemorrhages
to
clot
ap-
130,000/cu.mm.,
retractions
normal
and
the serum
prothrombin
7 per cent.
Splenectomy
was performed
on the 10th of August,
1953. The thrombocyte
count
immediately
preceding
operation
was 90,000/cu.mm.,
the tourniquet
test slightly
positive
amid
the bleeding
time 3 minutes.
The
spleen
was found
to be of normal
shape
but slightly
cmilarged,
weighing
250 Gm. The cut surface
appeared
homogensous
anti rich ins pulp.
Microscopic
examination
of the spleen
revealed
small groups
of Gauchen
cells scattered
in the
intersinusoidal
tissue.
No megakaryocytes
were
seen
ins the sectionus.
Six hours
following
splenectomy
the thrombocyte
count
had risen
to 160,000/cu.mm.
the
white
blood
count
to 15,000/cu.mm.,
and
the red blood
count
was 4 niilhions/cu.mni.
The
bleeding
time
was 1 minutes
and the tourniquet
test
was
negative.
After the diagnosis
of Gaucher’s
disease had been established
on histologic
examiiinat.ion
of the spleen,
the preoperative
bone
marrow
smear
was re-examinsed
anud a few Gauchen
cells
,
were
found.
of the
her
X-ray
lower
condition
repeated
cu.mm.
and
On
the
blood
normal
14th
the
tourniquet
and
there
patient
well
185,000/cu.mm.
the week
time
counts
cord
count
after
disease
coincidentally
was
absence
occur
destructiomi
the
cells.2
In
blood
of the
delivery
the
successfully
markedly
large
years.4
During
the
of
when
niext
two
140,000-200,000
e’
retractiomi.
ranged
showed
infant
fromu
of
2tX),(X)0
to
300,000
120,00()/cu.mm.
of Aimgust.,
was
1954,
the
185,000
thrombocytes/cum.mm.
was
80,000
amid oms the
mother’s
daily
platelet
per
found
patiemit
5th
counts
day
ranged
ENT
in cases
rate
and
the
enlarged
numbers
treated
amid
of Gaucher
is
present
in
et
in
in
al.
as
ascribed
these
patient
ivem’e
presemit.
organs
m’are, is
appear
Imi most
nianifestatiomi
adult
patient
In their
case
with
the
well as the hone
refers
to a sinuihar
as
althoumgh
late
in the coumrse
of such (‘ases amsemia
crowdimig
out
of the l)one
miiarrow
was mio anemuia
despite
the homie
of the femur.
omihy climiical
an
Wintrobe
to
there
X-ray
the
described
cells.
this
disease,
symptoms
is slow.’
by sphemiectomy.3
both
Gaucher’s
which
case
the
in
uu’as thromuonly slight
that both
other.
of progression
purpura
Davis
purpura
each
sphenomegaly
demonstrated
is rare.
to
feature
Thrombocytopenic
was
related
especially
and
outstanding
Gaucher
disease
clot
a minimum
On the 17th
thrombocytopenic
not
of marked
disease
an
by
and
and
to
the
counts
operations
of Gaucher’s
disease
is unusual
in that
its muaimi feature
purpura
imi the absence
of anemia,
leukopemiia
amid ivith
of the spleen.
However
the possibility
must
be comisidered
Gaucher’s
of
bleeding.
lesion
after
to 160,000/cu.mm.
This case
bocytopemiic
enlargement
known
and
thrombocyte
The
of
flask-shaped
week
wa examimsed
in the third momuth of pregnancy.
thnombocyte
count
was
220,000/cu.mm.
During
COMM
The
signs
one
thrombocyte
of pregnancy
when
tests
were normal.
female
baby.
the thnombocyte
During
no
bleeding
and
Enlenmeyen
discharged
revealed
test,
felt
characteristic
was
were
1954, the patient
phenomena
and the
a healthy
after
birth
130,000
patient
of February,
the
delivered
Two
days
the
The
examinations
until the 9th month
routine
hemostatic
cu.mm.,
from
showed
femur.
satisfactory
no bleeding
pregmiancy
and
of the
was
months
She had
examination
third
ins Gaumcher’s
this
liver
syndrome
ii’ho
amid spleen
w’ere
muarroiv
(‘ase
in
(‘omitained
a child
of 8
From www.bloodjournal.org by guest on June 16, 2017. For personal use only.
A.
Although
disease
sis
thrombocytopenic
is very
VRIES
AND
purpura
this
rare,
idiopathic
of
DE
disease
as
should
t.hrombocytopenic
G.
1057
IZAK
the
sole
mamiifestation
be comisidered
in the
of Gaucher’s
differential
diagno-
purpura.
SUMMARY
A patient
pumra,
The
bumt
with
110
Gaucher’s
amiemuia
hemorrhagic
disease
or
is described
leukopenia
diathesis
and
amid
platelet.
count
SUMMARIO
Es
describite
un
sed
(‘ytopemn(’
Le diathese
patiemite
iii anemia
comi
had
who
slight
thrombocytopemiic
enlargement
responded
pur-
of the
well
to
spleen.
splemiectomy.
mx INTERLINGUA
de Gaucher
morbo
mu leucopenia
hemorrhagic
omsly
qui
e solmente
habeva
umi heve
e he cont.o del plachettas
purpura
respondeva
thrombo-
del splen.
allargamento
hen a splenectomia.
REFERENCES
l3nocx,
of
2
M.
ANt)
Gaucher’s
FEmNBERG,
ROBERT
ansemia.
DAvms,
an
New
F.
W.,
inustance
WmNTROBE,
M.
L.
JACOBSON,
disease.
Acta
AND
0.:
The
England
J.
Med.
234:
A.
ANn)
SMITH,
M.:
Clinical
hypersplenism.
Hematology.
and
the
diagnosis
of
the
osseous
1: 165,
G.
QUIGLEY,
GENECIN,
of selective
histogenesis
Haemat.
C.:
527,
E.
1948.
Osseous
Gaucher’s
disease
with
macrocytic
1946.
W.:
Gauschen’s
Bull.
Johns
Philadelphia,
disease
with
Hopkins
Hosp.
Lea
& Febiger,
thrombocytopenia,
84: 176, 1949.
1952,
pp. 696.
type
From www.bloodjournal.org by guest on June 16, 2017. For personal use only.
1955 10: 1055-1057
Thrombocytopenic Purpura without Anemia and Leukopenia in Gaucher's
Disease
A. DE VRIES and G. IZAK
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