From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
The
Life
Hereditary
By
Span
Ehliptocytosis
Familial
G.
ARNO
and
Hemolytic
CROSBY,
VERNON
EREDITA1Y
tion
its toutitse
blood
elliptocytosis
Hereditary
with
such
fecal
a patient
urobilitsogets
(1)
disease;
cteased
cases
have
is that
may
itsvestigators8’1
divides
no signs
anemia,
12 per
classificatioms
the
cases
of hemolysis;
(2)
a “competssated”
beets
icterus,
hemolytic
agglutinat.iots
the life spats
atsd plottimig
of technical
obtaitsed.
Recently,
visual
technic
with
(Ashby)
elliptocytosis.
Agreemetst.
betweets
the
the piesemst itsvestigatiot1s,
ss’e transfused
families
and followed
survival
of the
sickle
sharp
cell
cototrast,
dat.a
in
other
hereditaty
Irons
the
t.lse
Research,
Michael
Submitted
*
The
term
atid
hemolytic
Depart
mcuut
of
the
dition.
The characteristic
(and “ovalocyte”)
will
general
atid indisease
to study
of ehliptothe disapdifficulties
Hedemistedt
the generally
oise patient.
D.
Imistit.ute,
will
place
the
Our
results
notssicklimsg
are
with
tramss-
eliiptocytes
compared
of hereditary
Medical
C.
used
imi this
red cell will be named
be used for oval red cells
57
Service
ausd
Chicago,
for publication
lie
With
tsvo methods
was not obtained.
elliptocytes
from three domsors
transfused
cells by the method
relatiots
Army
Washingtoms,
Research
heredi-
with
itsto
similar
elliptocytosis
to
is discussed.
Hematology,
2, 1953; accepted
“elliptocytosis”
to
coittstimog.
sytodromes
Center,
Reese
May
helped
facilitating
literature
Medical
Army
Reed
preparatiotos
thus
three
hemolytic
of diffemetstial
agglutimoation
as well as by a special
visual
method.
Patients
sickle
cell anemia
were selected
as recipients
its two itistances.
Followitig
fusioto,
of
itsto
of circulatitsg
red cells is abmsormally
short
to he reported
here deals with the life spats
to sustaits
Heilmeyer’s
classification.
data have
photogtaphic
of differential
condidiscovered
harmless,
useful
36
‘
He
have
attempted
to establish
blood
itsto tsormal
recipients
shaped
erythrocyt.es.
Because
the
abtsormally
method
hereditary
During
of differetot
is usually
have spletsomegaly,
reticulocytosis,
; (3) hereditary
elliptocytosis
with
of such procedutes,
tso consistent
and
Berliis’2
comhimsed
a careful
accepted
atsemia
with
t-ed cells,
conveustional
methods
to have
with
hereditary
elliptocytosis
recorded.1
no
hereditary
elliptical
conditiots
of Heilmeyer.7
AND
a l)enigts
and
by
beets
elliptocytosis
hemolysis
but.
ttat1sfusimsg
of the
the
demotistrated
hemolytic
als(l atsemia.
The average
life span
its arty hemolytic
disease.
The work
of elliptocytes
amod the results
appear
Several
cytes
by
pearatice
MC,
of oval
M.D.,
FSA,
USA
is oftets
presetsce
to Other
SINGER,
MC,
COL.,
CAIT.,
While
have
been
, 2 Severe
is itsft-etiuetst.,
l)ltt such
heteditaty
elliptocytosis
taty
the
by
Relationship
Diseases
KARL
LT.
S1ITII,
examitoatiotss.*
cetit. of all patiemits
its(-Iease(l
hemolvsis.t
categories:
Its
ELLIPTOCYTOSIS
matoifested
Elliptocyte
M.D.,
MOTULSKY,
H.
\VILLIA1
H
of the
the
Graduate
Department
School,
of
Walter
Hematologic
Ill.
June
paper
6, 1953.
t.o desigmuate
“elliptocvte.”
seems its maui’
the
hereditary
The term
“ovalocytosis”
types
of anemia.
con-
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
58
LIFE
SPAN
OF
THE
MATERIAL
Ashby
Survival
Whole
Time
blood
venesection.
from
the
after
transfusion
elliptocytic
Serial
Chicago
AND
METHODS
Studies
from
after
ELLIPTOCYTE
donors
counts
were
laboratory.’3
The
is taken
to
be
was
transfused
performed
inagglutinable
100
per
ituto
using
recipients
the
counst
withiui
method
two
previously
twenty-four
to
sodium
metabisulfite.15
days
described
forty-eight
hours
cent.
Sickling
Sickhilig
was
liptical
cells
elicited
stood
its stained
blood
such
are
difficult
cell
erythrocytes
cells
all
sickle
quite
be
films
distinct
made
from
to
sickle.16
1. M.
H.,
the
by
out
its
bacterial
sharp
of sickle
to
cell
anemia
patients
from
the
or
appear
true
sickled
oval
sickle
elliptocytes.
and
It has beets
cells.
and
but
elliptocytes,
characteristic
nonsickling
by
the typical
against
differelutiate
into
the
method’4
contrast
The
rod-shaped.
anoxia
shown
Ordinarily
tramisforms
oat-shaped
almost
fomnis
that
el-
red cells
Manuv
which
elliptocytes
are
cannot
Donors
Case
a 33 year
Walter
Reed
rapidly.
Rout.ine
to
and 2). The
elliptocytes.
tracer
utilization
of
to
this
blood
sister
family
labeled
treatment.,
the
arid
three
Ous
1-Summary
TABLE
red
was
paper
Women’s
cells.
and
the
ferrous
hemogram
were
Hematologic
interpreted
stabilized
of
Case
as shown
Elliptocytic
Donors
2, L. J.
Case
F
F
Race
Negro
Negro
White
Red
blood
cell
coutit,
(X
Pos
0
Rh
Pos
B Rh
Pos
N
Pos
A Rh
Pos
B Rh
Pos
M
106)
3.13*_4.6t
4.8
5.0
9.3*_13.lt
13.4
2.7%
Hematocrit
32*_40t
Reticulocytes
0. 1*0.5%t
3.8%
per
100 cc.)
3, E. B.
Rh
13.8
42
(GIn.
ouue
A Rh
0
Hemoglobin,
1.
as
42
28
F
type
its table
moved
33
blood
(previ-
ous
(Total
nunsber
Whit.e
blood
of
cell
reticulocytes)
coumit
Sickling
Osmotic
Serum
fragility
bilirubin
Fecal
urobilinogen59
(mg./24
hun. 4 day
of
responded
Sex
type
cent..
evidence
patient
hensoglobius
Data
1
and
complete
as
The
patient’s
1, M. H.
and
Age
Recipiemit’s
cleared
(tables
clearance
sulphate.
was
the
electrophoresis,
Case
Blood
admitted
elliptocytosis
plasma
findings
with
of Significant
was
which
blood
picture
with
many
ovalocytes
be tested.
Serum
iron was 55 -y per
These
months
Corps,
infection
anensia
accelerated
treated
Army
respiratory
significant
markedly
by the
after
upper
showed
a similar
members
could
patient
persisted.
of the
a severe
revealed
revealed
iron
and
lieutenant
for
studies
studies
deficiency
Elliptocytosis
old Negro
Hospital
patient’s
No other
Radioiron
iron
Army
bleeding)
(3130)*.(23000)t
(163,000)
(135,000)
5050*_7550j
10,200
9,750
Neg.
Neg.
Normal
N omnsal
0.3
1 .0 nsg.
68.6
mg.
mg.
190
nsg.
collection)
Hemolvtic
Survival
*
Before
t Three
the
itidex
(60)
(Normal
10-20)
time
anemia.
117
iron
admitsistration.
nsousths
after
iroli
administration.
30
1 14
days
Elliptocyt.osis
45
days
persisted
103
after
days
treatment
of
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
MOTULSKY,
SINGER,
2.-Distribution
TABLE
of
Degree
Least
(Red
cells
are
arbitrarily
Oval
of
Two
(see
III
shaped
E.
After
6
wit hi usornial
coluil)ousent.
l)\’ the
was
nset.hod
of
32%
8%
10%
6%
76%
34%
50%
meusomrhagia
,
withi
nsobilitv
due
.‘
multiple
ELLIPTICAL
ROD
SHAPED
I
5%
8%
1
13%
iron.
No
iticrease
denaturation.’8
to
routsd
Rod
IV
atud
c
55%
alkalitie
I Normal
groups:
OVAL
treatmemst
(at
III
0
3%
niouiths
four
erythrocytes;
ROUND
I
three
iuito
Elliptical
II
0
B.
Preparations
Counted)
erythrocytes)
I
M. H.*
L. J.
59
SMITH
in. Dried
Cells
discussion)
ervthrocvtes;
Patient
AND
Elliptocytosis
Hundred
divided
II
crythrocytes;
CROSBY,
iuu fetal
The
uterine
hemoglobinu
l)resumtth)le
fibroids.
could
source
be demonistrate(i
of
Hysterectomy
was
the
moms
deficiency
l)erformed
at
(late.
study
It shiould
be pointed
out
that.
the
red cells
of this
patient.
‘ere
transfused
before
iron
treatnsent
was started.
(‘asic 2. L. J. , a 28 year
old Negro
woman,
was seen at Michael
Reese
Hospital,
because
of amenorrhea
and
a possil)le
ovarian
cyst
. Physical
examination
was
There
was
mio jauusdice
atid
(VtOsis
was
nia(ie.
‘as
the
absemice
coumit
raluge
Case
treatnieuut
niarketi
of
rallier
thaui
was
explaiuucd
1).
old white
year
cells
Svnll)tOnlatic
increase
woman,
to
in
hereditary
the
the
elliptocyt.osis
per
in the
bilirubin
cc.
of ellipt.o-
of 3.4
amouuit
2850
ellipto-
degree
reticulocytosis
auid
was
l)rccedinug
fansily
could
of
(see
episodes
admitted
was
uterine
a1)seuice
ovalocytosis
1)y the
index,
method)
due
2) . The
in the
(table
i)lOOd
No
of
1. The
Chicago,
negative.
marked
extrense
this
of
fetal
cent
upper
hemoglobin
Total
blood
volume
was
to Michael
Reese
Hospital
for
spontaneously.
metromrhagia
clliptocytosis
roti-shuaped
atuti
hensolvtic
(table
revealed
of the
a diagnosis
in table
(Evauss-Blue
disappeared
1-. B. , a 42
3.
surgical
rioted
studies
In view
disease,
sunimnarized
elevated
volunse
cc . Amisenorrisea
4600
the
be
Plasma
are
blood
be examined.
hematologic
data
163,000),
Routine
could
of other
should
tletcctcd.
splenomegaly.
relatives
Hensatologic
(absolute
miormsial
or
2). No
(table
cytosis
a later
for
fibroids.
not
be studied.
anemia
blood
Routiuse
A large
suggested
The
discussions).
studies
tiumber
hereditary
nsiid
revealed
of elliptical
eiiiptocytosis
elevation
of reticulocytes
of bleeding.
Recipients
The
red
thoc blooti
cell
autcnsia
red
cells
rccipicuit
survival
the
validity
cells
of
of cases
who
case
1 (M.
2 atid
3 were
had
trauusfused
H . \V. lund
of the
1)eeuu
into
of
the
were
both
shortetsed
old
into
boys
cells
sickle
with
had
survival
(R.
It has
l)eniodicalb’.
with
( nansfuscd
dousor
transfused
8 ‘ear
trauisfused
patients
beets
Iuornsal
H.)
cell
anemia
a miormal
W.
and
survive
miornsal
blood
about.
found.
This
finding
time
of
J.’s
H.)
previously
l)eemi
L.
volunteer.
0.
(case
been
showms
red
in
this
tiormal
In additiotu,
atiti
to strengthen
cells
sickle
that
19
previously
hselped
of
classical
normally.13’
a year
2)
Recipients
with
normal
further
recipient.
RESULTS
Differential
Figui-e
Survival
Agglutination
Studies
1 shows
the results
of the red cell life spats studies
by the Ashhy
time of the tratisfused
elliptocytes
W’as normal
its cases
1 and
method.
3 (M. H.
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
(jo
LIFE
OF
SPAN
TIlE
ELLIPTOCYTE
x
x
8)
C-)
U,
U,
C
0
C
4)
*
U
4)
0
20
I0
30
f0
40
50
60
70
DAYS
Ftu
(\‘t
. 1
t I misc
Sutrvival
.--.
(Coui(.rol
0515.
r(’vealcti
mit)rluial
agglutiusable
couuut
37,50(),
1 .
nial
l)atieti(
atsd
E.
13
.--
couuut
11.-c.
:i.
circle
red
E.
, sit’klc
cell
ceuut
cell
laot.icuit
C.
ausensia
couuit
atsemisia
l)a(icu)t
II . ; XI
=
( any
ci 11pt oH.
\V . : 573
iuiagglutiuuahle
(lotion
W.
000
i mi-
cells-blauik
8,000.
H. \V. ; vliitc
\1 . I I .-rPci
(‘mdc
=
, muon-
l)i(’tit
.)
B.).
Thse
elliptocytes
of case
Thse
2, Isowevem-,
Ashby
curve
Isad
of about
siblv
slightly
totsvex.*
This is ititerpreted
as steady
(lecay
of this
red cells with all et-ythrocytes
havitug
an average
life spats
(lays.
:s1. H.’s
forty-five
days.
its this
a defitsitely
spats
lived
120
necipiemit
: L . J -It.
values
cells-biamuk
sickle
amuensia
cell
11. : 1 ,1S8,()00
iuiagglu(iusablc
L. .J.-retil)icnt
l)itt icmi( s vi t Ii huenedi
intl o sickle
B-C.
110
TRANSFUSION
froni
(‘5
tells
huustinetl
36,500,
: 320,0(X)
tlomior
r(-tipictit
tFt)ruuisl
t imise . ) ( )tue
(ells-1)lamuk
( Black
dotson
sutrvival
of
100
90
AFTER
pt ocyt
clii
t u-amisfuuu’d
of
I ramisfusiomu
80
ase
shortemsed
was
straight
life
or
patiemit’s
of about
pos-
shsot-tfort.’-
five
from
(case
rate
of med cell
survived
wete
protluctiomu
tsormally,
iroms deficietstt
will
give
it may
whets
rise
to
be
concluded
vithdrawms
hema-
umieveuu
they
ptesunsably
mildly
erythirocy(e
Siusce
cells
iron deficiemst
cells have a tuommal life spats. This is its keeping
with knosvn
tologic
amsd pigmemst
excretiots
(lata its pat iemsts with mild iroms deficiemuty.
Ats
domsor.
1) red
tisat
*
the
uuievemi
(list
nibut
ion
as
far
its
is cotucenned.
A prepo.tiderttice
of youusg
red cells
will lie found
iui (lit
I rauisfused
blood
if red ccli produc(
ion
of t hue domior
increased
shsort lv before
vcuucscc( 11)11.
The
Ashhv
curve
of such
blood
will he cotuvex sitice
a large
scgmcuit
of voutug
ervthrocv(es
will usot be ti(-st roved
itu the first few weeks
after tnauisfusion.2#{176}
t Although
‘‘age’’
(hue absctutc
of hiypochromia
on
nel)eatcd
examsuimsatioums
is muot explaiuieti.
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
MOTULSKY,
Fuu.
2.-Imiagglutiuiahle
followimig
Special
the
totiist.,
(\1.
attentiots
doimsg
the
elhiptocytes
trauusfusioui
while
M.
cells
the
It
should
chambet
on
the
couuit
the
cells
its the
for
cells
that
2).
day
after
with
A photograph
after
was
tratssfusiomi
were
13 per
presemsce
blamik
approximately
day
about
The
chamber
recipietst’s
Elliptocytes
only
eighty-secotsd
counting
the
agreed
(table
recipient.
rod-shaped.
day
ous eighty-sccousd
elliptocytes.
eighty-secomsd
normal
or
Ashby
donors
oms the
l)e recalled
SMITh
Allowimsg
various
0I
AND
to
elliptocytic
the
elliptical
of
tests.
cisamber
itsto
of
poitut
shape
of the
1)
were
countitsg
to
chamber
Arrows
of inagglutinable
coumstimsg
2).
(fig.
Flu.).
agglutitsatiots
(case
erythrocytes
CROSBY,
on
C.
paid
of elliptocytosis
of the
certsible
was
numl)er
obtaitsed
II.’s
1.1. to
differetstial
degree
SINGER,
cetst.
of this
disdotsor’s
of elliptocytes
transfusion
of
clearly
in the
is therefore
sig-
tiificatst.
‘vIild
t-eticulocytosis,
hsemolytic
itsdex (table
process
its the l)atietst
l)resetit.
was
These
compensated
out..
It
dense
tise
L.
its the
1) further
with
the
corroborated
dimitsished
finditsgs
hemolytic
has
meats
life
total
1)een
(55
stroyed
daily
per
Normal:
state
the
this
patient
t-ate
one
=
0.09
t.f
was
14.1
Gm.
hsemoglobin
prO(lucitsg
cats
out
the
utsirshibited
normal,
to fift.eemi
Gm.),
the
per
productioms
al)Out
2t
equals
marrow
atiemia
to eighteeti
0.25
Its a steady
much
may
days),
per
Gm.
inwhen
Knowitsg
(forty-five
Kg.
de-
of hemoglobits
chronic
hemolytic
of (lest.tuctiots;
hemoglobin
wellcarried
occut-s
days.
time
that
as
are
bone
atid
=
Kg.).
times
of recogtsizitsg
of hemoglobin
of hemoglobin.
Isemoglobits
elevated
hemolytic
iso amsemia
determinatiotss
survival
amoumst
amid the
of a mild
However,
difficulty
time
ats
(635
ramsge,
existetsce
time.
over
is reduced
calculate
Gm.
isormal
the
survival
survival
tisat
hsemoglol)in
Kg.),
(‘
showmi21
cells
upper
to poitst.
uisless
6 to 8 times
of red
circulatitsg
atsd weight
Kg.
output
spats
hselp
disease
recently
hsemoglobims
l’s
a bilirubin
therefore,
as
usormal.
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
62
LIFE
3.-Percentage
TABLE
of
Tra
(Initial
Day
SPAN
count
after
OF
Sickle
Compared
: 200 Elliptocytes
per
33
62
65
41
39
62
47
43
59
57
25
44
76
20
28
90
10
15
explaitss
in
Sickle
3 summarizes
occurs
parallel
of the
results
two
do tsot itsdicate
at atsy
time
duritsg
was
compensated
survival
of “isormal”
the
obtained
However,
the
Ashby
the
atsy
cetst
visual
intervals
coutsts,
techtsic
significaist.
3).
elhiptothe Ashhy
ideustifiable
technic
chatsge
after
the
l)ut
(table
morphologically
coutstiisg
(E.
of elliptocytic
at various
to the serial
with
5 per
by
that.
their
elhiptocytes
tsumber
methods
w’as obtained
with short-lived
study
no transfused
cells remaimsed
by
be ideistified
results
This
by coutst.itsg
its sickle
cell preparations
line could
isot be fitted
day.
still
of atsemia.
Preparations
as obtaitsed
the
forty-fifth
could
abseisce
results
recipient
erythrocytes
A good straight
ots the
elliptocytes
Our
Cell
the
cell
the
rough
check of the
L. J. Its L. .J.’s survival
of
100
89
78
a sickle
techtsic
of E. B. ‘s inagglutinable
on Ashby
count
cells
80
roughly
cytes
Percentage
75
C8ounts
A similar
agreement)
86
Elliptocyte
values
rough
15
productioms
1000
Note
26
disease.
cells per
tratisfusiots.
cells.
Following
Count
Percentage
of E. B. ‘s elliptocytes
in suckle cell preparation
I
100
92
Increased
in
Preparation
Ashby
1000
8
hemolytic
Table
Cell
with
2
B.)
ELLIPTOCYTE
Elliptocytesnn
nsfusion
transfusion
THE
at
in shape
that
time.
of elliptocytes
life spats.
DIscussIoN
Hereditary
Elliptocytosis
Erythrocytes
arbitrarily
vs. “Symptomatic”
from
itsto
patients
four
groups
normal,
round
erythrocytes
(IV) rod or cigar-shaped
careful
and
atsd
various
the
for
established
diameter.3’
There
appear
affected
; (II) oval
erythrocytes
such
hereditary
dependitsg
calculatiotss
mathematic
have
w’ith
Ovalocytosis
the
erythrocytes
(table
2).
to estimate
based
subdivisiomss
; (III)
European
exactly
ots
may
degree
the
be
divided
of elliptocyt.osis.
elliptical
(I)
erythrocytes;
authors
have
degree
devised
of elliptocytosis
measurememsts
of
red
(-eli lemsgth
22
to be
families,
wide
variations
so that
its the
iso
proportions
categorical
number
of oval (II), elliptical
(III),
a diagnosis
of hereditary
elliptocytosis.
elliptocytosis
A sigmsificant
elliptocytosis
upots
atsd
mttst
be carefully
distinguished
itscrease
its the usumber
of oval
of ellipt.ocytes
statemetst
rod-shaped
(IV)
This
is importatst
from “symptomatic”
atsd elliptical
red
cats
red
be
made
its the
as
to
cells msecessary
since
hereditary
cells
ovalocytosis.
occur
may
its
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
MOTULSKY,
many
comsditiomss
where
“symptomatic”
SINGER,
atsisocytosis
ovalocytosis
is practically
always
of a pre(Iomitsatsce
of hereditary
cells
that
are
tomatic
other
drop
cells,
ovalocytosis.24
itistances
of
Recemitly,
an
associated
the
more
irois
W’ith
itsfec-
of elliptocyThe
presetsce
favors
associatiomi
the
diag-
of deformed
itsdicat.ive
with
tary
hemolytic
be
sigtsificatst
of
symp-
a
super-
evets
clitsical
and
hematologic
the
isumber
to
make
the
case
judged
be
must
family
elliptocytosis.
and 13 per
elliptocytosis.
(groups
III
portion
of the
red
W’ith
of extremely
of oval
case
IV)
red
in case
cells
nature
cell
are
owts
be
definite
Survival
striking
oval
b.lood
family
of a givets
disease
study.
The
the
subdivisiots
other
(groups
elliptocytosis
as
cotsditiots
with
evidetsce
(group
It should
(group
was
similai-
II).
may
a true
case
of hemolysis
III)
atsd
These
elliptical
Time
shape
Studies
of the
films
obtained
or
of
with
(case
sitice
2)
the
had
(group
red cells were
erythrocytes
that
instamsce
of elliptical
rod-shaped
II
\‘aty,
of
heredi-
patient’s
atsd
the
IV)
rod
(76
proproof
sister
had
forms.
largest
cells
morphologically
its the other
a large
defimsite
The
percemstage
per
cemst
atsd
itsdistitsguishable
two patients.
It
of the red cells is atsy index of their
tsot suggest
that
there
is a correlaaisd imicreased
hemolvsis.
in the Literature
elliptocyte
photogtaphs
these
he isoted
this
In
available
tsumbers
suggested
workers.
Several
investigators8”
and followed
their
disappearatsce
values
ots blood
heredi-
true
differerstiate
questiotsed
tiuestiotsable
whether
the degree
of deformity
viability
simsce large-scale
family
studies4
do
tiots betweets
marked
degree
of elliptocytosis
The
hemolytic
hemolytic
extensive
hereditary
1 is itsterest.ing.
of the
cetst respectively).
similar
oval ats(1
to early
patietsts
disease
studied
merits.
3 might
mildly
anomaly
oval
Elliptocyte
comsgetsital
ovalocytosis”
betweets
usually
cells
of
ovalocyto-
The very
large
tsumber
of oval aisd elliptical
cells (50 per
cetst respectively)
its the absence
of anemia,
suggests
true heredThe rather
small
isumber
of elliptical
atsd rod-shaped
cells
atsd
familial
same
fitsditsgs
spheroactually
hemolytic
have
been
tsonspherocytic
diagtsosis
ots its
study,
atypical
without
thats
may
symptomatic
had “symptomatic
differential
diagtsosis
impossible
Sitsce
isecessary
patieist
disease,
to splenec-
be more
sti-iking
by spletsectomy
with
hereditary
and
amsemias.
Without
amid
difficult
develop
favorably
of cases of cotigetsital
red cell shape
atsomaly
hemolyt.ic
elliptocytosis
quite
characteristic
of the
atsy
may
respotsd
spherocytosis
number
Some of these patietsts
Its occasiotsal
itsstatsces
the
may
IV)
spherocytosis
27-s)
3)
every
hereditary
itscreasing
iuonspherocytic
films.29
listed
hereditary
may
cured
(hereditary
time
strotsgly
etc.-is
Sitsce
ovalocytosis
its such
patietsts
reports
of hemolytic
elliptocytosis
atsemia).24
ietst’s
associated
occurrence
IV)
Suds
severe
comsditioms.
hatsd
patietsts
26
8 per
from
(group
promitsetst.23
anemia,
hereditary
Such
represent
sis.25,
the
Ots the
w’ith
symptomatic
tomy.
cytosis,
tary
cetst
itary
cells
elliptical-tear
patietsts
Occasionally
to
is
its pertUcious
of the
deformed
63
ovalocytosis.
imposed
not.
SMITH
poikilocytosis
ittscommots
cotsfirmatory
ellipt.ocytosis.
isot
AND
amsemia,
amsd severe
aisemia
A demotsst.rat.ioms
of family
of very
nosis
atsd
is not
deficietscy
anemia,
Cooley’s
tiomi, leukemia,
atscl camscer.
tosis
CROSBY,
of
methods
its use
tratssfused
by periodic
such
ratsged
for survival
elliptocytes
itsspect.ioms
preparatiotss.”
from
Red
twelve
time
studies
itsto normal
of the recipcell
to thirteets
survival
days9
is
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
64
LIFE
(from
cases
with
thirty-four
the
Sitsce
the
sample
following
a
atsd the above
data
The first survival
tratssfusiois
formed
ss-ho
of blood
from
is
with
90 per
studies
usimsg erythrocytes
was straight
with
normal
,sic
yielded
four
3).
1 atsd
ats expotsetstial
days.
These
postulatitsg
of elliptocyt.es.
both Ashby
our study.
glut.irsable
cells
Relationship
and
this
hemolytic
diseases
recetit
itsformation
trast.ed
as sickle
morphologic
anomaly
cyt.es’3
“
mally.32’
and
hereditary
of the red
spherocytosis,
cell associated
alone
atsd
does
target.
msot imply
decreased
cells from
pat.ietsts
elliptocytosis
shaped
pattertss
sickle
with
pareists
of patietsts
for
pathologic
for
With
sickle
the
sickling
or
getse
results
its
hereditary
elliptocytosis
ceist)4 amotig
the populatiots
Negroes)
atsd thalassemia
homozygosity
*
Patiersts
for
with
severe
elliptocytosis
hereditary
nomnsal hensatologically
and
the hereditary
spherocytosis
anemia34
(“trait”
be
spherocytosis
clitsically.
Doubtful
getie or nsutatiors
etc.)
as
such
is a
Shape
trait
et-yt.hrosurvive
msom-
Similar
to
minima
the
cots-
pat.iemuts,
the
disease
pathogenesis
thalassemia
disease
its spite
of the
A
its both
less
major35
are
double
dose
itistances.
frequetstly
(0.04
the genes
for sicklitig
some
Mediterratseats
predicted
to occur
are
to
ktsown
patertiit.y
nsay
cots-
its diseases
sickle
cell
mitsima
respectively.
is distributed
may
hereditary
of
heredi-
as that for sicklitsg
atsd heredas a metidehiats
domiisatst.4
Both
or
getse
at large than
(4 per cetit of
of
rfable
4 summarizes
hereditary
hemolytic
disease.
its the
hemolytic
of hereditary
group
iso hemolytic
for elliptocytosis
by both sexes
cell
betweems
its a case
the
t.halassemia
have
thalassemia
the
3).
finding
atsalogous
importaisce
by
of
aisd thalassemia
major
with shortetsed
life spats.
atsd
elhiptocytosis
red cells.
are of great
spats
itito
life spats since
with
thalassemia
trait
etsd
Syndromes
characteristic
is an
cell
sixty-
techmsics
agreemetst
(table
matsifestatiotss
atsemia,
deformity
the
spats
shape
amsomaly.
of the various
cell
its
the
life
The
about
isear
rough
securely
notsatsemic
at
shape
Hemolytic
cell
litse
two
life
Hereditary
elliptocytosis.
hemolytic
diseases.
The trait
spherocytosis*
is itsherited
zygous
gene
mild
of patients
abtsormally
Genetic
tary
itary
their
observed
majority
their
more
base
its
tech-
utsder
the special
comsditiotss
of
the expected
tsumber
of imsag-
erythrocyte
cotiditiots
(as
photographic
the
itsdicate
hereditary
Hereditary
ditiomss
data
The
elliptocyt.es
betsveets
tiormal
atsd
photogt-aphic
elliptocytosis.
the
the
techisic
showed
associated
with red
on the pathophysiology
atsd
with
to Other
of dimitsished
places
syisdromes
our
throughout
Similarities
demonstration
elliptocytosis
reachitsg
itsto
contrast,
with
per-
survival
Berlims
atsd
with
was
day
recently
of tratssfused
discrepatscy
and visual
coutstitsg
our Ashby
couists
to be elliptical
a patietst
to
blood
is iisaccurat.e
tets
Very
results
elliptocytic
Its
method
Similarly,
the
method
agglutinatioms
spats
curve
explained
from
elliptocytes.
experiment,
twemsty-six
recipient’s
to omse hutsdred
from
(cotscave)
authors
a chatsge
life-spats
The
same
visual
a
spats of such erythrocyt.es.
with the Ashby
method
differetstial
life
to
its
atsy
life
cent
simultatseous
Its the
small,
otse hutsdred
survival
Ashby
cases
sytsdrome)
elliptocytes
the true
of elliptocytes
reported
our
of
very
reported
a patieist
ELLIPTOCYTE
hemolytic
HedenstedV2
time
curve
TFIE
isumber
utsderestimate
time study
Trllsick3’
OF
“compensated”
days.’#{176}’“
by
SPAN
exist
arid
be itivoked
where
to explaits
of
the
Sitice
the
to 0.1
per
(10 per cemst of
populatiotss),6’
rarely.
incomplete
hetero-
Omse such
both
parent.s
expressivity
these
cases.
itsare
of
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
MOTULSKY,
stance
was
reported
daughter
by
of patetits
tiemst
(the
case
one
atsemia
was
its the
tiois.36
A few
semia
major37’
hemolytic
to
may
elliptocytosis
span
with
of the ellipt.ot-yte.
a sharply
dimitsished
The
getse,
red
carriers
view
mild
of
recetst
theory
could
cell fragmetsta-
also
foutsd
in thalas-
Other
spherocytosis,
cases
atsd
by Holst.-Larsets5
paretst
carried
the
data,
atsd
trait
by
for
for
the
It
dimitsished
always
is foutsd
for the
E
red
cell
hematologic
is usually
C
sickle
carriers
course
aisd
is possible
the
that
thalasIts
other
of
heter-
39
survival
picture
explained
by
hypothesis
combimsation
cell
causes
(table
life
be associated
its thalassemia
minima).32’
of the
of
mark-
time
postulat-
getse.
The
a
as
to
of the
trait
and
the
getses
a distinct
disease
caisnot
hemolytic
mimsor
detected
sicklemia,
electrophoretiby
elliptocytosis,
(table
with
getse)
of
C is an
be
sickling
thalassemia
variatst
Hemoglobin
4)42
“expres-
for
syndrome
kisowts
as microdrepanocytosis
cell trait
(arriers
(heterozygous
sicklimsg
hemoglobits,43
methods.
sselI
heterozygous
ams alterisative
from
a mild
are
variable
respective
itiherited
hemoglobits
distinct
#{176}The
be suggested.
by
who
or elliptocytosis
genetic
as
W’Ould presumably
This is exactly
what
shortetsitsg
of the
a hemolytic
mat.itsg
of sickle
The
tsormal
moderate
may
characterized
tologic
,
(thalassemia
of thalassemia
heterozygous
(ally
are
osmotic
instamsce
to red
is outstatsditsg.
with
itsdividuals
to
, produces
41
fragility
tsormally
mimsor).32’
thalassemia,
pareist
due
fragment.atiots
associated
of most
petsetramsce
petsetramsce”
4).41.
he
survive
(thalassemia
imsg imscomplete
Its
cells
patietsts,
ozygous
stt-ikitsg
Homozygosity
life spats.
however,
heterozygous
is fouisd
osmotic
its this
itscreased
its this
fragmetstatiots
defitsite
and it was also presetst.
in siblitsgs
of the patietits.
of I)acie
amid of Holst-Larsets
imsdicate
that
heterozygosity
of
semia
itscreased
the
pa-
fitsdimsg
markedly
probably
w’as
The
had
A noteworthy
and
was
patietst
hematologically)
jautsdice.
osmotic
fragility
have
beets published
co-workers.24
Its these
cases otsly one
gemse
majot33’
examined
atsd
etythrocyte
with
Their
elliptocytosis.
I’slicrospherocytosis
illusttatioms
elliptocytosis
Witsship.4
microspherocytes
aisd
65
SMITh
noishemolytic
and
elliptocytosis.
whete
AND
atsd
marked
childrems
of
spherocytes
ellipt.ocytosis,
The cases
atsd
five
published
edly imicreased
Dacie
atsd his
had
spletsomegaly
presetsce
additiots
its
be seets
CROSBY,
Bancroft,
1)0th
of
with
the
ftagility
Wyatsdt,
who
omsly
hemolytic
SINGER,
usual
hema-
its cases
its which
only onse pareist
has elliptocytosis,
may represetst
the combitsatiots
of the elliptocytic
trait
with atsother
gemse which
canisot
he detected
by orditsary
clitsical
atsd
hematologic
examitsatiotss.
It is of itsterest
that
elliptocytosis
has beets reported
in associatiots
with
the
sickhitig
phemsomeisoms
Although
the
pemssat.ed
of thsis
The
globimi
hemolytic
patietst
synthesis
was
isot
of
tary elliptocytosis.
hsemolytic)
case
by
Of great
further
ahmsormal
others.46
to
itsterest
family
data
studies
givets
is the
do
fitsdiisg
were
carried
isot
exclude
that
the
out.
a com-
elliptocytes
sickle.
getsetically
our
determitsed
abnormalities
uisderstatsditsg
of the
of sicklemia’8’
hemoglobins
electrophoretic
normal
adult
Alkalimie
No
the
clarified
atsd
Paper
1 revealed
pat.ietst.’6
l)e made
of specific,
has
of thalassemia
imsvestigatiots
same
msot. atsemic,
process.
could
demotsst.ratiots
festatiotss
reported
its the
patietst
denaturatioms
(table
are
4).
begitsnitsg
to
of hemo-
various
Methods
be applied
used
its
study’7
of the hemoglobims
of our
hemoglobims.
Similar
flmsdimsgs have
tests
for
the
detectioms
of fetal
matsiits the
heredi(msotsbeets
hemo-
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66
LIFE
SPAN
OF
THE
ELLIPTOCYTE
E,
-
H
:
e
r12
.‘s
.
C
i
,
o
n,
C
le
C:C.
‘,
-‘ri
.
C.--
-
)
1
Qs._:
:
a:
:
s_
I
.
:.._
fi
2
C
2
::2
ij
-C
-e
.5
‘
‘
fi
:
.
a:.=
.2
C
E
.
.
‘..
0
C
:2Ea:Ca:
Ca:
cI2--Z.C
Os
2
Os
C
C
.2.j
.9.;;i:
a:
c.-o-.
b
C
C
=
a
C
-n
I
C
C
.2
C
.C
a:
0
n
C
.‘
C
C
Cl
-
Cl
Cl
Cl
C.’
C
1
C.’
C.
‘
3
C
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
MOTULSKY,
SINGER,
CROSBY,
AND
SMITH
a:
-
C
C
.C
a:
-
-
-_
a:
a:C
CC...
C
z
Cl
CC
ZE-
C
C
.
.
Cl
a:a:C
a:-
ClClCl
C
Cl
Cl
a:.
a:
C
L
Cl
ClClCl
E
Cl
:r.’rrj.’
=
C.
I
i
C
E4
a: Cf..’ C
C
a:
lb
t
Cl
‘2
CCl
Cl
Cl
,Cl
‘1
Cl-h
_
........C
.
C)
Cl
fi
Cl
C
.
Cl
.C
C
C
C.’.CC
Cl
C
C.
C
‘
I
‘-j..
C
-
C
be
Cl
Cl
I
$-
Cl
a:
Cl
CC.C
bL
C
-
C
a:
...-
a:
a:a:
C
Cl
be
a:
C’)
C’)
C
-
t Cl
I
I
Cl
a:
a:C
.Ei
.C
Cl2
C.
-
Cl
‘2
Cl
C
c
‘
Cl
C
be
.
C)
.9
Cl
C
.E
a:
.‘C.
Cl
ClCl
‘-
.
fi
Cl
Cl
a:C..
c2 C
C.
a:
-C
C.-
E-
fi
67
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
68
LIFE
b8
revealed
case
Iso
isormal
additional
as its five
of
further
values
patiemsts
hemolytic
are
elliptical
first
three
cells.
The
momsths
ELLIPTOCYTE
1 atsd
2,
tsotshemolytic
has
as
(case
3 was
tsot.
elliptocytosis.
yet
beets
studied)
as
To our
studied
well
kmiowledge
electrophoretically;
required.
of hereditary
Itsfatsts
with
other
at
hereditary
birth
have
svith sicklemia.49
Otsly a few red
proportion
of fetal
hemoglobits
cell
cells
hemoglobits,
whether
the
almost
atsd
as
has
cramsial
patiemsts
hereditary
do
tsot
Treat
may
cats
its t.halassemia
the
characteristic
It is tsot. yet
is related
to
atsd
in hereditat-y
elliptocytosis.51
abmsormalities6’
ellipt.ocytosis.
tsucleated
that
the degree
do not support
to sickle.
mitsor
its hereditary
maxillo-facial-detstal
show
be made
its elliptocytosis
occur
reported
with hereditary
spherocytosis,
suggested
Our results
life.47’
phetsometsots
beets
and
of
all red
amsalogous
atsce of fetal hemoglobits.
Mild erythrocytosis
cytosis
tsumber
elliptocyt.osis
to the
hereditary
ellipt.ocytosis
of elliptocytes
gradually
imscreases
durimsg
the
48 A similar
phetsometsots
occurs
its itsfatsts
cells sickle at birth.
As erythrocytes
wit.ls a large
become
replaced
by others
that
cotstaiti
sickle
to
four
TilE
its cases
with
climsical similarities
are of itsterest.
few
OF
elliptocytosis
itsvestigatiotss
Some
syisdromes
SPAN
the
spisero-
Botse
chatsges
beets
described
have
such
its
Leg ulcers
have also beets sects.52’
As its
red cells atsd reticulocytes
its elliptocytosis
shape
atsomaly.4’
of elliptocytosis
such hypothesis.
For
increases
this
with
reasots,
agitsg
it has
of the
beets
red
cell.
inent
No
treatmemit
comsditiots
hemolytic
lance
is required
is betsigts
elliptocytosis
duritsg
atsd
imsfectioti
for
by
also
whets
patietsts
botse
destructiots.
beets successful
elliptocytosis
this
where
with
marrow
its all
operatiots
was
fitsdimsgs
teristic
are
sigmsificatst
of notshemolytic
ciated
simsce
where
may
cases
performed.3’
In
it was
fail
54
The
Competssated
careful
surveil-
to keep
pace
of hereditary
24-26,
‘
hemolytic
As discussed
osmotic
all cases,
meistiotsed,
fragility
al)ove,
is isot
elhiptocytosis
remaitsed
charac-
atsd
the
operatiots
evems though
the patieists’
red cell values
returtsed
Sitsce the hemolytic
mechamsism
of ellipt.ocytosis
is utsktsowms,
is available
to explain
why splenectomy
betsefits
these
patietsts.
It is quite
however,
due
spats
of
stroyed
by
patient
with
at least
the
the
part
of the
improvemetst
“fragmemstatiots-spherocytes”
spleets.
thalassemia
The
of spherocytes
which
favorable
also
by the
was
had
beets
after
to tsormal.
150
imsformat.ioms
likely,
to iiscreased
selectively
de-
respotsse
major55
“fragmentatiots-spherocytes”
removal
hemat.ologic
asso-
or itscreased
the
that
with
true hereditary
spherocytosis
cat it sot
Osmotic
fragility
its all but two of the
imscreased.26’
Its otse of the patients
appeared
followitsg
the
operatioti.26
imscreased
elliptocytosis.4
microspherocytosis
eight
elliptocytosis.
sigisificatsce.
except
for
production
symptomatic
ovalocytosis
associated
with
be ruled
out irs three
of these patiersts.25’
26
eight
cases subjected
to splemsectomy
was
with
tsormal
fragility
matsy
spherocytes
These
tsonhemolytic
itself
is of tso medical
requires
iso treatmetst
coistitsued
itscreased
Spletsectomy
has
life
ktsosvti
disappear-
to spletsectomy
of ats occasiotsal
h)e explaitsed
its a similar
matsmser
sitsce
may
occur
spleets
its this
is the
basic
disease.37’
hemolytic
38
It is well
mechaisism
kmsowms that
of heredi-
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
MOTULSKY,
t.ary
spherocytosis.56
a isormal
Its
SINGER,
Hereditary
57
life spats.58
view
of the favorable
should
be
Careful
study
performed
AND
cases
absence
of spletsectomy,
of
patietsts
red
cell
that
time
spleen
the
hemolytic
survival
amid after
spletsect.omy
will cotstribute
much
taty
ellipt.ocytosis
aisd hemolytic
mechatsisms
of the
w’e feel
“decompensated”
with
69
SMITh
its the
spherocytes
effect
its
of such
CROSBY,
have
operatiots
ellipt.ocytosis.
determinatiotss
before
to further
utiderstanding
its getseral.
of heredi-
SUMMARY
cells
Red
1.
cytosis
from
differemst.ial
Life
3.
A life
4.
spats
of forty-five
who preseisted
Its two recipients
W’as
techtsics
was
survival
cally
itsdistitsguishahle
was
evideisce
sickle
by
There
shape.
from
families
with
followed
heteditary
by
the
the
elliptocytes
ellipto-
Ashhy
techisic
of
basic
no
with
number
hemolytic
have
of
results
elliptocytes
of the
yet
respoissible
the
were
visual
ellipto-
motphologi-
life spats.
beets
for
atsd
from
elliptocytosis
isormal
its sickle
Ashby
of traissformation
With
of a third
its the abseisce
of atsemia.
the survival
of the tratssfused
the
evidence
elliptocytes
defect
with
the
betw’eets
patiemsts
cases.
of hemolysis
(-eli atsemia
ahtsormalities
The
its two
obtaitsed
countimsg
was
from
Isemoglobin
ellipt.ocyt.osis.
isormal
days
agreemetst
obtaitsed.
imst.o tsormal
Elliptocytes
was
followed
Rough
cytic
5.
Nt)
other
with
also
preparations.
6.
of differeist
their
of elliptocytes
spats
elliptocytes
cell
patieists
atsd
agglutitsat.iots.
2.
pat.ietst.
three
tramisfused
were
demotsst.rated
hemolytic
its hereditary
elliptocytosis
remains
umskmsosvms.
7. Symptomatic
ovalocytosis
differentiated
8. The
lytic
tic
place
diseases
atsemias
patterms
As
from
of hereditary
9.
elliptocytosis
(table
major,
explaits
Nt) treatmetst.
ectomy
is advised
the
1. Erythrocytas
esseva
success
of the
for
techmsica
Ashby
2. Le durat.iots
3. Uts duratiots
the red
closely
atsd
operation
atsd
tres
pat ieistes
tratssfundite
e br
be
of familial
hemo-
hereditary
hemo-
osmotic
hemolytic
after
tsomshemolyt.ic
fragility
due
elliptocytosis.
splenectomy
Issmay
ellipt.ocytosis.
ehliptocytosis.
Splen-
elliptocytosis.
INTERLINGIJA
de
differeiste
supervivetstia
familias
observate
cots
elliptocytosis
per
de agglutitsation
differeistial.
de vita del elliptocytas
esseva
tsormal
its duo casos.
de vita de quaratsta-citsque
dies esseva
obtetsite
pro
cytas
de uts tertie
patiente,
le absemitia
de amsemia.
must.
life span
(atsd getsetic)
foutsd
its thalassemia.
itscreased
of
hemolytic
IN
cell
that
other
its hemolytic
hereditary
“decompemssated”
de
to the
may
occur
its cases
“fragmemstatiors-spherocyt.es”
SUMIARIo
hseredit.ari
of aisemia,
classificatiots
similarities
microspherocyt.osis
is required
for
types
its the
4) atsd
are poitsted
out. It is showts
that
of hereditary
elliptocytosis
resembles
tell fragmetstat.ion
life spats of these
partially
its many
elliptocytosis.
is discussed
its t.halassemia
to red
creased
occurs
hereditary
Ic qual preseistava
altere symptomas
medio
del
be ellipto-
de hemolyse
its
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70
LIFE
Its
4.
duo
recipietstes
elliptocyt.as
cyt.as
cots
traissfumsdite
del
imsdicatiots
de
OF
de
techtsi’a
THE
atsemia
esseva
its pteparatiotses
resultatos
SPAN
a cellulas
etiam
cellulas
Ashby
falciforme
sequite
per
falciforme.
e del
traissformation
ELLIPTOCYTE
del
be supervivetstia
comstar
le
Ums accordo
techmsica
forma
visual
elliptoitst.er Ic
approximate
esseva
elliptocytic
del
de
isumero
al
constatate.
forma
Nulle
tsormal
esseva
ol)servate.
5.
Elliptocytas
memste
ab pat.iemstes
imsdistimsguibile
6.
tsqi.te
hemolyt
ic tematse
Ovalocytosis
distitsguite
de
Le loco
8.
familial
sigmsalate.
es
“sphetocytas
9.
hereditari
debite
iste
Nulle
typos
demomsst.rate
pro
its
ellipt.ocytosis
de amsemia
e debe
ha
a
operatiots
iits
esser
post
tractameisto
similissime
pote
de
augmentate
pote
duratiots
splenect.omia
a illo
e
erythrocytas
augmetstate
its elliptocytosis
oct-utrer
de
in
vita
de
its
parte
explicar
iste
le
hemolytic.
es requirite
“tsots-competssate”
(-otsfiguration
del
I.Tn
hemolyt.ic
amsemias
hemolytic
es
del erytht-ocytas
e etiam
microspherocyt.osis
fragmetstation
hemolyt.ic.
de morbos
heredit.ari
de vita
majot-,
fragmemstatiomsal”
de
essite
its le classificatiots
thalassemia
elliptocytosis
successo
ha
responsal)ile
its multe
hereditai-i
its
osmotic
morphologica-
tsom-mal.
hemoglobina
fuisdametstal
occut-re
elliptocytosis
fragilit.ate
esseva
vita
hereditari.
(Ic elliptocytosis
Como
de
de
discutite,
e similit.ates
al altere
Ii vs motsst.rate
que its re dutatiots
thalassemia.
Pto
incogtsoscite.
symptomatic
hemolytic
durat.iots
de
defecto
elliptocytosis
getseticamemste,
casos
de
atsormalitates
. Le
hereditari
osis
cots ellipt.ocytosis
elliptocytas
isulle
isutic
elliptocyt
7.
de
pro
elliptocytosis
hereditari
elliptocytosis
hemolytic,
notshemolytic.
spletsectomia
es recommetidate.
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From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
1954 9: 57-72
The Life Span of the Elliptocyte: Hereditary Elliptocytosis and Its
Relationship to Other Familial Hemolytic Diseases
ARNO G. MOTULSKY, KARL SINGER, WILLIAM H. CROSBY, LT.COL. and VERNON SMITH, CAPT.
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