1. health and fitness
2. disease

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HISTORICAL
Paroxysmal
A Classic
nocturnal
svn(Irorne
1 1(1(1 anol
191
such
a l)tt ient
iii
in 1866,
Strul)ingm35
in
the
new
entity,
the
citation
ease
ft-oni
1882.
afldl
characteuized
INtSe(l
io)i15
i)itP(’i
\Vt5
has
0)1’
fulls’
denouistuated
cited
the
iii
believed
an(I
‘
‘(lit-o)ni(’
hid’tD(
‘‘.
.
l’ronii
I loslat
well
that
andi
sixty
ihe
later.
andl
a
by
the
dliSof
a(’ouracy
of
Although
Str#{252}-
case
othel’Wise
as
was
theories
exceptional
been
Paul
regal-died!
dlisease
proposed
years
re-
case
by
be
the
ol)seu\’ations.
a somewhat-
l)tol)osedl
)gl( )h )iIill
t’it-hi(’fll(
of cold!
com-
almost-
t li(’
Ziskinid
I
I ivisli
,
)V
grant
record
of t lie
fn’otn
ml aine(l
Library
s
\Vashiingt
ol
fronii
t lie
m\
t banks
on,
t
I).
i\
ones
found
the
1\I ichuehi
nuen-
in the
I )onat
‘ ‘
has
litera-
Ic anemia
amended
i
stuck.
t lie
) of
amid
it le
observations,
hemolyt
type.
(hleniatology
al
his
epo)nynl
vho
that. ‘‘
‘splenonuegalic
‘
the
audi
\Iau’chiafa’a,
propose(1
he had
iafa’a
ane-
of hemo-
hemosideritutliiIt
published
Lttld’li
l’
an(l
u’hich
(hisease
,
( ‘emit me I lospit
of t he
.
rnii
to
for’
in
t lie
Medic:tl
I’niiversit
I)r.
( liarl
C..
itles listed
t lie
Milan;
auul
amounts
,J. II . Prat
1)eiatn’t
miiemit
of
t I)iagnost-ic
\Eechicinie,
Tufts
8(11001.
to
majon’it
lahorat
lnigl:tnid
1928
in
entity
cases
the
lon’intmtia
ty)d,’’
lse:trchi
Nei
)d(’(hi(’:tl
;ided
Lii macv
t lie
l)atieu1t
henolyt-ic
‘acquiue(1
‘
I))’ hat-ge
I)el’Petual
sinuiliau’
calling
)5i(
of
repo)rtedl
disease
with
-ie\’et’al
\Itt(i1iItImt\’a-\liOh1(’li
.
uvtts
anemia
a ca-se
chau-Iictetizedl
a new’
seo’ondl
tinue
sanue
uepo)ut-edl
case
second
id
:: I io’lieli
t1 anal
(‘ohlege
hd’fllOlVt
flue
07
#{176}-
uu’it ii
Forghiere,
clearly
it- has
was
ioleuutified
\Iiux’Iuiafa’a’s
at
Nazat’i
vhuich
A
hue ha(l
stuidiod
tiouuing
type,’’
utine.t
that
dtlle(l
this,
in
igluo)redl.
sidet-in
t uut.e.1
excellent
dlescription
of his patienttests,
he dhiffet-entiated
some
luem()gloh)iuultia,
Marchiafavadlescrih)ed
syn(lrome
might-
subject
clinical
as
an
(lefinite
a
hemoglobinui-ia
of his
called!
first
been
published
as
u-ecogniZe(l
occasionally
nau-ciu
mimt., \\i(lmtl-Al)imlflhi
t he
analysis
19 1 1 , \lau’chuiaft-a
In
latet
.
A.U.S.
have
Gull65
l)au’oxysmal
and
l928.#{176}Actually,
the dhsease
u’as
f-hat Charles
Stewarttuu
diescribed
it by a careful
136 B’ provocative
ltl)Oti
heeuu
henoglobiuuuuia
I)letelY
cases.)m
of
entity
in
possil)le
lie
M.(1.,
to
first
uecognizel
papeu
on the
Strul)ing’s
in 1882,
commonly
\Villiam
u’as
investigation.
of eaulier
olcoituct
I)ings
Althouigh
o)thieu’ fou-ms
pathOgO’uU’siS
It is eveuu
olisease
of (‘linioal
classic
his
I 793.
(oL.,
helievedi
a clinical
as
Li’.
hemoglobinuria,
generally
l#{176},)th
century.
1)ort
a
is
red’o)gnizedl
in the
knouvu
Hemoglobinuria
I-I. CRosulY,
WILLIAM
AHOXYSMAL
)iIid’hlCII
Nocturnal
Description
by Paul Strubing
a Bibliography
of the Disease
By
P
REVIEW
I.uis
Itunid
ani(l
he personiniel
t heir
count
Chicago;
Marval,
t he
(/
of
esv
bibliography.
I ibran’v
v of
(he
t on
t
nor’
arid
t hose
t he
aut
atice
270
Aires;
in
lick,
amid
providing
(‘onicernie(l
Dr.
Jean
ion
Army
1)ivisionn
public:ttionis
lion’
niiversitats-Bibliot
Buenios
my l’ounidat
1)ai
Reference
assist
For
from
I I . I loud
.
t lie
which
Medical
the
mm! with
could
riot-
be
I am in(lel)te(lto
Got tigeti
; l)r.
Reniato
,
Favre-Gilly,
Lyon.
From www.bloodjournal.org by guest on December 22, 2014. For personal use only.
WILLIAM
From
time
to time
instance
has
been
the
to
describe
first
the
right
disputed.2’
ft has
disease,
of the
been
was
271
CROSBY
of paternity
H
.
the
H.
Italian
protest-ed!
not-
the
who
have
reviewed
Marchiafava
and
to it in their
paper.)
Hmans
van den
hemolytic
Bergh
process
was
anism
in vitro
was
Biffis8 published
patient
occurred
patient
was
in Holland,
and suggested
the
literature,
Nazari
w’ere
reported
due
only
aware
a case
to a defective
potentiated
by carbonic
a case report
in 1915,
only
in the
evening
Calabresi’7
made
in 191
He
cell
and
acid.
noting
or at
the
noted
the
common
many
cases
had
when
been
the
in America67
mans
slight
van
been
found
Almost
in the
oxysmal
and
that
which
vork
which,
f-he
mech-
hemoglobinuria
also
pointed
in his
out
that
his
taken
Dacie
up
was
15,
45,
66,
scant-
he
red
the
cell
the
studied
have
had
clinical
picture
There
have
hemoglobinuria,
All
syndrome.
not been
it been
hitherto
published
upon
by
after
constitutes
146
until
the
latter
in Holland,5587
the
finding
of Hy-
normal,
hemolysis.
a normal
globulin.28
and
that
It has
plasma
The
conremark-
u’as invoked
to explain
the
sleep the respiratory
(‘enter
increasing
hemolysis.
a theory
dlesirable
identified
today,
129,
f-he plasma
in increased
of hemolysis
now’ been
reported
and f-he disease
It- seems
117,
confirmed
accelerator
and
89,
by Jordon
is acted
coagulation
68,
only
thrombosis
attention
independently
in England.35
appreciated
f-hat
the red cell is dlefective,
plasma
in vitro
results
defective
‘
case
have
evolved
at least
123 cases
has become
a w’ell
to
call
attentid)n
to
the
of parknown
early
and
especially
td) review
Strubing’s
w’ould
be regarded!
as a significant
*
Other
globinuria
that
hemolytic
hemoglobinuria.
thrombosis
of acidity
on the hemolyfic
mechanism
of hemoglobinuria
at night.35
67 1)uring
and carbon
dioxide
is retained,
thereby
contribution.
history
and
may
piecemeal,
cases
the
idlen-
iefeuence
metabolism,
demonstrated
by changes
in the plasma
Neither
Marchiafava
for
It is now’ recognized
was
that
of the
the
which
70
20th
century.
nocturnal
much
*
68
‘
acidification
‘
venous
in this disease.5’
6, 7.
the hemolysis
received
prol)lem
Bergh
den
of nocturnal
of
published.
threat
of
Ham
st-itut-ent69
able effect
occurrence
is depressed
phenomenon
occurrence
most serious
The
mechanism
1930’s,
not
similar
to that
of Marchiafava
and Nazari.
In 1925, Enneking47
unaware
of the Italian
reports,
published
a carefully
studied
that the disease
be called
“paroxysmal
nocturnal
hemoglobinuria.”
had
The
this
ol)hque
demonstrated
the
He
noted
an
that
that
night.
In 1927, Scheel,’28
in a study
dealing
with
pigment
that
f-he nocturnal
hemoglobinuria
was accompanied
hemoglobin,
l)ilirul)in
and
urinary
uroi)ihnogen.
Micheli
has
of it and
red
was
to
that
neither
he nor Micheli
wrote
a definitive
Chauffai-d
and Troisier24
in 1908 had published
w’ho three
years
later
was described
by Marchiafava
authors
in this
Marchiafava
i-ecognize
it as an entity
diescription.
a i-eport
of the same
patient
and Xazari.
(Of the many
first
and
tity.
hematologists
that
early
of
reports
nocturnal
was
that
of ParoxYsmal
henioglobinuria.
reporteol
hienioglobinuria
The
in 1832
by
first
Johns
are
idenitifiable
Elhiotson,
of interest
case
of
of lonidon.nui
as background
paroxysmal
The
for
cold
disease
the
hemoits
this
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2 72
early
As
as
published
I 794,
an
thra.”
It
The
em-ises lasted!
became
crisis
was
much
of any
the
in
spade
only
appearance
‘iVilhiam
s’ere
IV. (
in
leaf-her
practicing
at Archangel,
of blood
from
the
had nocturnal
with systemic
a l)u-anny
though
of
and
sedliment-
regularly,
to) tinge
the
red!
at- the
the
colour,
second!
mention
made
u’as
first
d!ischarge,
less
severe
uu-as very
urine
made
time
was
of the
ure-
hemosympof fulness
crises
the
A
.
if- returned
continuing
of a deep
No
of making
perfectly
influence
free
of cold
nor
or chills.
of Lond!on
About
in flue
exciting
reported
an incontestable
time
a numheiof cases
that
English
cause
who,
showed
: instead!
days,
blood.”
uill6i
18(16.
w’orker
cold
case
ordls
discharge
l)ut
‘
(‘olouued;
of
published!
“The
variety.
quantity
of fever
globinuria
surgeon
periodhical
hemoru-hage,
of three
slightly
association
color’
: “The
diminished
the
a Scottish
patient,
a 51 year old Russian,
three
days and were associated!
his
‘of natural
‘
L HEMOGLOBINURIA
in the had’k and lower abdomen,
torpor,
and a sense
severe
crises
the urine
was dlark all olay. Between
dlescrihed!
it- was
Stewart,
of a singular
that
urine
NOCTL’RNA
L
(‘haties
‘Aco’ount
‘
t-oms, such as pain
in the head.
During
during
YSMA
is possible
globinuria.
if,
BOX
PA
in
literature.
was exposure
the
course
Gull
uvet
f-hat- his case was
‘i’he patient
and!
w’o)rk,
hemoluemoglobinuria
of nocturnal
cold!
of
believed
td)
his
d)f
case
of this
uvass a
cold.”
frequently
was
(!renched
uvith
w’ater Sd) that
he became
u’et- to) the skin.
Gull gives
an exceptionally
fine
history
and!
olailv
notes
of the patient’s
hospital
course.
Through
these
recthe pid’tut-e
of no(’turnal
hemoglobinut-ia
emerges
clearly
(lespite
the er-
FOflCOli5
case
was
It
\u’as foinud
to
Perhial)s
Because
(lue
of the
seetiis
to
to
“While
noted!,
contain
the
that
haematin
a cold
response
unilikely
ani
uu’as
diagnosis.
morning
hieniagglutinin,
quinine,
diagnosis.
this
for
case
Teniporar’
u-urine
f-hat
was
passe(!
which
was
passed
at
the
attacks
is usually
were
written
iniprovememit
in
prevented
off
as
of typical
f-lie
one
etrly
or during
noon
by
quinine.
This
malaria.
cold
of
paroxysmal
hemo-
glohinuria
(agglutiniimi
type)
under
large
(loses
of quinine
was
demonstrated
years
later,
1873, h)V Rohsert
I)ruitt
, an
l’nsglishi physician
i’ho ‘as himself
afflicted
by the disease,mtt
Elhiotsoni
reported
oh)servations
in his patient
ivhich rather
ehiminiate
malaria
as a diagnosis.
“This
nian was labouring
umider frequent.
chills,
but had not regular
aroxsnias
of ague.”
Elliotsoni
also S1)Oke of the “aguish
fever.
.
which
he suffered
whenever
the east wind
blovs,
or
he
is eXpOse(I
to
cold
ani(1 vet
three
attacks
in a single
(Ity.
I)espite
l’iliotsonm’s
I)riority,
l)arxYsniial
descript
iomi
scril)e(l
a 10 year
mal
cold
crises.
vithi
of
ho’nioglobimmuria,
I)ressler’s
careful
Tens
and
years
1)ressler
ivhi() was
since
is a niiucli
rel)eate(1
physical
mi England
later
his
ProIablY
exaniinations
a spate
of
an(l
cases
the hiterature.m49
\Iost
of these were cold
henioglobiniuria
reported
by
Gull was among
England
to the
(‘onitinemit-,
anid durinig t he niext
hemoglol )i nuria were reported.
In
many
of
absensce
of red
grounds,
Gull
pignuent-at
ion
the
cells
earls’
in t lie
suggested
of
the
reports,
therefore,
“haernatinuria.”
urine
in
all
of
cases
the
in
agglutinin
original
1S54.
type
nose amid ears
ol)servat-ion
the
had
during
than
He
de-
of paroxys-
hemolytic
Elliot-soni’s,
urinialyses.
paroxysmal
them.
Interest-
thirty
years
1868,
apparently
with
published
hemoglobiniuria
hemoglobinuria,
used
In
these
of
was
hemoglobin
and
urine,
with
patienit
credited
was
yanois
of the
piece
of clinical
better
the
is generally
report
afflicted
lie developed
report
occasion
of WUrt.zburg
hemoglobinuria.
child
old
Oni one
....“
mistaken
the
Popper
represented
but
in
almost
for
term
appeared
the case
the disease
1(X) cases
of paroxy’smnal
I)ressler
1)100(1.
“chromaturia.”
proposed
found
On
that
hcmoglobinuria.mum
in
of nocturnal
spread
from
the
the
paroxysmal
an
same
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WILLIAM
H.
273
CROSBY
the latter
part. of the day was healthy,
and so it continued
for as many
as eight
(lays.
Such
is the usual
clinical
history
of these
intermittentattacks.”
Stolnikow’
of St. Petersburg,
Russia,tm36
pul)hished
one of the early
reports
in
1880 but made
no mention
of f-he nocturnal
pattern
of the hemoglobinuria.
However,
on
one
in color,
it- is noted
day
containing
:
‘
The
a quantity
urine
accumulated!
of uric
dluring
salt
acid!
hut
no
the
night-
formed
was
elements
red
[casts
or blood
cells].
Thete
w-as a faint
oxyhemoglobin
spectral
absorption
st-ripe.
The
morning
portion
of the ui-inc was a deep straw’ coloi and! showed
no hemoglobin
in the spectroscope.”
Stolnikow
usually
collected
all of his patient’s
urine
each
olay
a single
as
i)inuria
was
specimen,
missed.
hemoglobinuric
so that
He
crisis.
did,
On
the
nocturnal
how’ever,
the
set
tenth
day
of March,
he notes,
for instance,
“temperature
The
pallor
of the mucous
membrane
yesterday
is very
ness
The
and
headache
fender
in the right
during
the
a murmur
at f-he apex
and!
Stolnikow
noted
exposure
his
that
patient
w’ith
the
right
dluinine
cold
hemoglobinuria
and!
something
to
pei-iod.
in 1880,
that
suggested
w’ith
did)
II.
bloodl
day
of the
crisis,
enlau-gedl.
The
liver
tongue
is fuzzy.
Borborygmi
20. The heart- uu’as enlarged,
cc.
of perfectly
nothing
effect.
to
black
wit-h
d!id not
the
He
although
he believed
kidlney
d!isease
as an
that
his patient
efiologic
factor
was
in f-he
the
he noted!
might
(‘Oncept
for that
have
pai--
jaundice
w-hich
an enlightened!
published
a uepoit
offer
urine.”
crisis.
be-
of Lyon
He
do
a diagnosis
dlestuilcfion,
Lepine9m
third!
is dlistinctly
1500
had
without
‘
Also
spleen
jugular;
to
yond
‘paroxysmal
hemoglobinuria,”
not a “typical
case.”
He eliminated
ticulai-
the
evening
38.8; this morning
37.4.
marked,
as it- uu’as yesterday
; weak-
to percussion
though
not enlarged.
The
lower
ahd!omen.
Pulse
88. ltespiiations
was
t-ieated
night.
chai-acteristic
of the hemoglodlescripf.ion
of a typical
a good
d!own
of a case
in which
the
typical
pattern
of noctuu-nal
hemoglobinuu-ia
is mentioned!
: “It is only at nightf-hat- f-he urine
was blood-colored
.
ai-ound
1 1 : 00 o’clock
or midnight,
the urine
was l)loodl-coloted
and! not the otheu- specimens.
The coloring
uu’as, moreover,
as
.
I have
said!,
onstu-ate!
not
intense.
an
colored
are
positive.)
of hemafuria,
dlilutc
jouity
of
cases
is the
only
six
the
ui-inc
in the
My
.
.
.
examination
cells.
1ed!
entiu-ely
.
ft-ce
seveial
.
.
u’enal
is the
patient
There
tul)ules.
result
had
are at
The
times
urine
He
of cold.
by
the
an
kidney,
concluded,
But-
i-epeafed,
d!emwhich
au-c
(They
were guaiac
specimens
of hemoglobin.”
is in i-eality
that
hemoglobinuria
dells are lost as such
ted
[f-he cuisis]
cause.
hours.
of
not-
contended
that
flue veuy
micioscopic
absence
probably
Lepine
in l)ed
On
absolute
.
“In
w’e need
unusual
and!
form
aie
lysed
flue immense
not- l)elicve
in
ma-
that
this
his paroxysms
at midnightuu’hen he had been
least two d!istinct
types
of paroxysmal
hemo-
globinuria.”
Paul
wright
*
Strubing*
uu’ho
Paul
reported
seen
his case in 1882’;
the patient
was
in Novembet-,
1880, in flue Medical
uu’as first
Strubimig
was
borni
in 1852 and
s’a1d in 1876. Ins 1889, he became
in 1900, professor
of internal
subjects
as
laryngospasm
and
director
medicine.
ozena.
took
his medical
of the
His
nose
later
and
degree
at the
throat
cliniic
published
work
a 29 year old caitClinic
of Professor
Uuiiversit-y
at
was
the
of Greifs-
University
confined
and
to
such
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274
PAHd)XYS.\-IAL
NOCTURNAL
service,
(lt-enchuings
“Flue
especially
uvith
of an overhuentedi
in
observed!
Mosleu
1876,
he
O’O)lOF change
up
and
by
The
enlarged,
l)inous
urine
flue evening
urine,
he
nate.
pain
the
neigluborhoodl
pain
in
the
liver
fote
the
eyes,
area,
at-tacks,
time,
this
his
extraordinary
fatigue
on exertion,
d!eveloped!,
well
and
.
.
.
w’etc
he
was
he
urinate
voided
once
or
pathological
The
most
constant
increased
palpit-ation,
after
habit-s
complaint
by
was
percussion
; lesser
sometimes
a glitter
be-
After
an attack
the
exhaustion.
a
very
severe
attack
w’cie
very
regular,
once
there
is a
daily
; after
in 24 hours.
attack,
36.6
to
which
and
and!
1880,
on.
hemogloday or in
.
accustomed!
which
vertigo,
stools
.
not
percussion.
dhisfurl)ed
without
any
urge
to) unhe had in the morning
naturally
cor-
at-tack.
bow’cl
2-3
an
b)etween
His
muscled
liver
from
November,
He void!e(!
(kirk
was not-ed! d!uu-ing the
.
fol!owing
of breath,
ears,
to
pu-od!rome
spleen,
shortness
w’ith
was
his
to) percussion
f-here
tempeuature
At
.
getting
Clinic
unless
which
the
tender
daily.
nights
of
in the
h)ladd!er.
association
tint
This
after
no lymphad!cnopat-hy,
Medical
examined
those
of
is tend!er
in the
“In
the
till morning
the symptoms
buzzing
area
se\’ete
on
intensity
f-he
kidney
and
at
of
in
pressure
enlarged
uu’as
ditlieflY
w’ifh
easy
black.
d)
passc(!
colou.
strength
to an
a w’ell
tongue,
urine
night,
intensity
physical
records
only in flue mot-ning.
No
before
an attack.
Although
the
responded
his
titine
yellow’
of exhaustion,
a normal
treated
the
slept-
The
pallor,
was
(luring
usual
a yellowgiay
spleen.”
examination
so)mcwhat
time
the
dIal-k-brown
the
in
of the
dlusky
spleen
looked
morning
lost-
primarily
physical
with
this
twice
the
assumed
patient
“The
sometimes
in
attained!
the
1)uring
flue latest
neighborhood!
man
.
urine
itself
ShOwed!
at
d!etailcdl
young
l)d)diy
his
he complains
in the
to)
had!
Now
pain
himself
tuaccs
his present
illness
to his milithe heavy
i1usicai
exertion
and!
the many
After
his d!iscluarge
fuom the military
service
negaud!
had bed’ome
pale and!
the past year
hue had
noon
“During
olegree.
that
OfllV
(‘omplexion
any
patient
of (iueifsuvalol.
tary
HEMOGLOBIXURIA
the
and
patient
37.2.
No
never
felt
eui.iption
a chill
of hives
was
or
heat.
The
observed
us’it-h
at-tack.
“\Vith
of o’old
in
the
J)iuning
heatedl
regau-d!
cOuld!
summer
the
Strubing
oluring
the
uvete
as frequent
just-
light
praying
bath)
pi-ecipitated
that
noted
subsequent
dlescnihed
ately
after
by
Fleisehuen-
the
‘‘behino!
Ot.lr
exercise
But
back’’
attacks,
(!osed
just
or
wore
but
himself
and
a dhrect
that
severe
as
those
in
the
open
air
at
bodly
w’ot-k
with
overwot-k
.
ether
u’ouulo!
was
salts
.
w’ithout
attacks
the
winter.
with
over-
noting
any
of giving
an
produce
come
effect
the
(insteao!
these
w’hich
“disagreeable
often
there
with iron
.
between
hemoglobinunia)
excess
himself
go into
as
would!
he
atta(’ks
stated!
he distinguished!
(march
Alcoholic
noctut-nal
he
his uu’hole
no aft ad’k.”
heavy
night.
exertion.
which
ind!ivid!ual
patient-
and!
winter
clothing
effect
of the
cause
The
00)1(1 of last
in the
thiosse
l)teoipitate
patient
pn-ecipit-ating
demonstrated.
be
severe
body
(letnimental
him a cold!
to) the
not
attack
episodes
emotions”
could!
no discernible
cause.
and!
pro)d!ucco!
ano!
immedhi-
on
an
also
The
attad’k
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WILLIAM
unequalled
nocturnal
in severity
hemoglobinunia
Strubing
the
studied
w-hich
has
lasted
been
his patient
H.
four
noted
diligently
275
CROSBY
to five nights.
in several
later
and
described
(This
effect
reports.t6’
77
the
of iron
82.
,
significant
in
141)
facts
about
disease:
After
1.
that
was
especially
severe
at-tacks
blood cells were dissolved
a penetrating
observation
argued
about
ing’s
.
-
1911.
Marchiafava
Free
in
found
urine
the
sediment
in the
were
urine
found
except
‘as identified
plasma
believed
contemporary,
Lepine
(above),
Although
he failed to identify
a fine-grained,
yellowish-brown
2.
“.
in
the
was
the blood vessels
for the time. The
He
that
construed
in the
this
kidney
as
or the
evidence
urine.
of hemoglobinuria
hemolysis
occurred
in
the
This
was still
kidney.
Strtib-
believed
that hemolysis
occurred
in the urine.
the pigment,
Strubing
described
the hemosiderinuria:
detritus
and fine-grained
casts
of the same
color.
yellow-browni
a rare
red.
and not
mechanism
in
one
after
renal
a very
epithelial
severe
attack.
cells.”
The
No red cells were
hemoglobin
in the
by spectroscopy.
StrUbing
pointed
out that
glohinuria.
The only way that
in the dlevelopment
sleep was the (letermining
factor
hemoglobinuria
was to be found
3.
in other
than
of hemomorning
urine
was by having
the patient
wakened
at some other hour. He reasoned
that a dissolution
of
red cells took place during sleep; that the process was a slow and gradual
one,
for there
were
never signs of violent
hemolysis
such as chill, fever,
and flushing;
that the hemoglobin
was
excreted
by the kidneys
into the urine. His further
reasoning
is made more interesting
by
our recent knowledge
of the mechanisms
of this disease.
He postulated
that the red cell
must be defective,
the product
of a disordered
blood-producing
organ.
He knew
that
all
red cells are somewhat
sensitive
to the hemolvt-ic
action of carbon dioxide
and suggested
that
these
slowing
Particular
acid
(from
the
hemolysis
of
patient
acid
manner,68
ducing
cells
the circulation
of
previous
these
and
might
be
during
sleep
(lay’s
abtiormal
sodium
He
hemoglobinuria
even
but was
enough
was
Were
accumulation
would
cells.
did not. give
senisitive.
anud the
exert-ion)
red
bicarbonate
but- Strubing
nocturnal
abnormally
provide
the
attempted
to
unsuccessful.
acid.)
This
not
heard
of
this
so,
he
of carbon
proper
acid
provoke
concluded,
dioxide
the
and
lactic
environment-
a crisis
by
for
giving
(A crisis cani 1)e produced
theory
of nocturnal
acidity
again
unitil
1937,
fifty-five-
the
in
this
proyears
later.35
67
4. Strilbinig
speculated
alout
the spleen.
At this
time
a conniection
between
the spleen
and abnormal
hemolvsis
had not l)eens accept-ed.
“Because
of our uncertain
knowledge
about
the function
of the spleen,
we niay only hi-’1)othesize
about
the s)lenoniegaly
and
its Part
Hi our
syn(lron7ue.
A close coninection
between
the individual
attacks
and the spleen
is provc(l
by
the apearansce
of Pains iti the splensic
area
after
ani at-tack
and
after
several
attacks
the appearance
of a slight
but definitely
Pall)al)le
enilargenient
and
increase(l
tenderness
of the spleen.”
5. \Ians’
patients
i’it Ii noct urnial
henioglobiniuria
develop
serious
t-hrombot
ic phenonuienia at some
time
(luring
the course
of their
illness.
Although
this
did
not happen
to
Strubinsg’s
Patient
, he
suggested
that
the
nianiy sniptonis
which
develo1)ed
Iurinig
this
attack
might
“Senisationi
chest
,
time
of the
symptoms
breath)
stroma
the
cough,
Landois,
be
due
iveakniess,
aini
attacks
mi
to
such
the
the
t-o the
and
to the
concerninig
a process.
exhaustion,
area
number
bowel
liver
arid
inconnpat
He
short-ness
of the
spleeti
of stools
which were due to anemia
and those
which
were due
in the
blood
might
somehow
circulation
chest),
soniehow
of
and
to some
the
symptoms
a sense
a1)doniinial
from
StrUbing
loss
(pain).
transfusion
t-o t-he
His
lungs
crisis:
in
Furthermore,
cells
during
(weakness,
(cough
based
dogs.#{176}5
and
thie
shortness
oppression
of
mi
on experiments
The
violent
the
the
hetweeti
suggested
that
free re(l
and a partial
obhiterationi
was
in
of a severe
apprehension
distinguished
of red
reasoning
react ion
of
pain.
other
process.
He
a formation
of fibrin
(hyperperistalsis),
spleen
ible
noted
1)reatli,
is increased.”
resulting
cause
of
cell
of
the
by
picture
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276
PAROXYSMAL
ivhichi
occurred
Lanidois’
in
aniinuals
to be a magnifications
Strubing
the
animals
ol)st
ruct
were
l’
ed
have
to
fibri
The
.
HEMOGLOBINURIA
(hienuoglobinuuria-,
of the
founid
t lie
NOCTURNAL
process
some
vahidit
ins his
arid
mesenteric
St ri) hing’s
The si flll)le headache
whirls
nnost pat ient 5 W t h
their
attacks
occasionally
progresses
to
cerel)ral
casionally
becomes
a full-blown
portal
thrombosis.
of red cell stronnata
or to sonic
other
mechianisni
are plagued
1)5’ thironsibosis
of a minor
and
nuiajor
hyperpnea)
lie ol)served
their
of
of
y
(hiarrhea,
which
speculat
aniply
doubt
degree
,
is
abdominal
this is due
but the fact
without
vessels
borne
out.
with
experience
The
Whether
to
nsortein,
blood
been
hiemoglobinuria
thrombosis.
is in
Post
I)ulmonary
ions has
nocturnal
appeared
patient.
cramp
oc-
to the development
that these
1)at-ients
question.
COMMENT
It is seen
from
the
luemoglobinut-in
above
accoutut
aco’ut’ately
was
that
svndhud)me
the
olescnibed
fifty
nocturnal
of h)aioXYsmal
years
it
before
was
accepted!.
differentiated
his dhsease
fu-om other
types
of pau-oxysmal
hemoglobinuu-ia
w’hich had been idientified!
at that
time.
He identified
it w’ith other
cases
of the same
svn(!rome
descu-ibedl
by other
authors
uu’ho had not recognized
the
significance
of their
(!escniptions.
How
then did f-his d!isease
fail to be accepted?
Strfibing
We
have
examined!
answet-,
the
writings
mu(’h
\Vithd)tlt
paroxysmal
had!
been
in
identified!
as
a(’d’epfeo!
Gull’s
somewhat
kid!nev
function
as
of malaria
in spite
of the
d!iscussid)n
is given
to
( mau’ch
hemoglobinuria
theories
regarding
ma!
that
mentioned!
(Old
was
!)
carbon
at face value
Stolnikow’s
Chvostek
.
however,
almost
gave
and!
.
of quinine.
with those
fascinated!,
uvas
dlioxide
this
Lepine’s
theory
of d!efcct-ive
case is d!ismissed
as a case
of feveu’ and the failute
case, which
is identified!
StrQbei-g’s
an
ieport
Chvostek
and! remarked!
very
He dliscussed
Lepine’s
in
hemoglobinunia
kid!ney
dhsease.
a
for
a comprehensive
review’ of
all of the cases
(almost
100)
each
of those
which
we have
hemog!obinuuu-ia
this
al)send’e
contemporaries
StrQbing’s
I-fe revicw’edl
an(!
fulls’ but accepted!
the huemoglobinuria.
for
of
published!
J)auoxysmal
tho’orv
moi-e
1894.
dlafe
noctu
ease
dO.5C
arid
to
paroxysmal
to Gull’s
as
briefly
pUl)hisheo!
many
Chvosteknut
hemoglobinut-ia
w’hich
of
sti(’d’ess.
a page
The longest
of Fleischer
by
to
Struberg’s
theii
description
d!iseasc
entity
(lisd’Iissid)Ii.
the
Perhaps
tinue
u’hcn
first
is
Stt-(ibing’s
name
seems
careful
work
dhiseases
foi
himself
vain
for
clinictm57;
has
of medical
this
plished
too
mitch.
Masiusnt)
thirty
This
before
iecognizc
his
may
d!isease
disease
dlefect
than
luereditary
immoutalifv.
It
know
remains
synonymous
nocturnal
to) two) causes.
name.
works.
it,
flue
with
hemoglobinuria
a mou-e
These
u’as
first
Belgian
has
toget
f-hat
w’e look
from
Biermer’s
pernicious
anemia.
peu-haps,
titled!
described.
observers
her
achieved
cases
serious
a
Strfibing’s
little
or,
by an ad!eduately
spherocytosis
anemia,”
mattets
among
at
The
Bestowing
While
by lumping
pernicious
uve nd)w
Minkow’ski.156
good
Biermer,
“progressive
is shared!
this
laid!
he
a dlistinn.’ti\’e
attention
it might
have
caughton.
is doubtful.
The
work
contains
in whichi
years
as
anemia”
his
to
his d’ontempouary,
them
anenuia,
huaol called
luemoglobinut-in,
give
unnoted,
“Biermei’s
w’o)rld
dlesctil)ed
of focusing
naming
a measui-e
even
1871,
gone
med!ical
a(lequiatelv
to
way
anol
But
and
first
failuie
pel-nicious
Strubing
nesic
it was
a sui-ei-
several
of’ the
failut’e
the
flaw-it
work
in
If
aphyp-
accomof Vanlair
This was in
named!
their
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WILLIAM
disease
“microcythemia,”
But
it didn’t
onstrated
catch
red
normal
spherical.
spherical
cells
by
proposed!
way
“red!
cells
on the
due
to destruction
only
was
a new
was
advanced!.
disease
but
it was
backedi
was
either
ignored
fifty
Masius:
It
years
to
or
water
the
patient’s
scale.”
an entirely
observation
elapsed
before
seems
intellectual
well
indeed
must
be
blood
rival
claims
to
its
d!iscovery
between
1915
Yet-
this
w’ith
work
Vanlair
almost
leap
they were
declined
to
iigid.
For
a theory
the
right
climate,
Given
1927
of hemolysis
in three
find!ings
in 1882, it may
and
the
disanewitness
simultaneous
labot-atoi-ies
be said that
about
1936.
the climate
not- propitious.
was
St’uiut
1 . The
tention
raphy
2.
huisfot-v
of
pau-oxysmal
fh
outstanding
w’ork
of
l)articUlau
to the
attention
earhieu-
dhifferentiatcd
the
disease
which
part
of
disease
obscu-vat
of his
basis
repou-ts
huave
ions
now
played
nocturnal
from
the
in the
Strubing
as
is
is ie’iew’ed,
and
centut-y.
A bibliog-
StrObing
identified
19th
reviewed!.
a dhisease
entity
in
flue disease
with
great
1882
but
at-
failed
to
calling
accuracy,
by sleep
in ptecipitating
the paroxysms.
hemoglobinuu-ia
and
by pI-ovod’ativc
tests
d)thiem paioxysmal
hemoglobinunias.
On the
he proposed!
been
hemoglobinuria
published
Paul
luemoglobinuria
name.
He described
cited!
BY
noctuu’nal
is called! to fouu- case iepotfs
of the d!isease
is appended.
1)aroxYsmtil
noctut-nal
give it a dhisf-mcfive
He
too
and
w’it-h them.
right.
and
cells,
due to that
at that
time,
appear
in several
places
at once.
Witness
the recent
simultaneous
covery
in several
laboratories
of folic acid,
the vau-iants
of Mediterranean
mia, and the Hh antigens.
Or, coming
back to noctui-nal
hemoglobinunia,
development
of the mechanism
When
Sti-ubing
reported
his
were
of disease
red
it. may
the
them
and anemia
one paper
not
same
fault- ; in one
the contemporaries
ordicred,
“climate”
up
make
concept
the
so it- was
caught
possessed
the
lnhappily,
injuring
patients
jaundice
In this
experiment.
Strubing,
p1-ogress
both papers
contemporaries.
also
there
u’as jaundice
was entirely
new’
and!
w’it-h
As
that
in their
pnimai-ily
dem-
Masius
show’ed
new’ nosologic
involving
designation.
and!
w’ould
found!
i-ed cell dlestruction,
jaundice.
All of this
clinical
they
cells
a grand
“on
a d!isease
rejected.
of ideas
f-hat
cells
hut
w’ith
by
or
yeaus
distilled
a catchy
Vanlair
blood;
spheu-ical
and
red
present-ed
up
flue
the
paper.
patient’s
adding
that
of excessive
hemolytic
evolution
flourish,
in their
dealt
is evident
that
ahead
of their
leap.
The
cells
even
a remai-kable
to destruction”
rfhis
because
i.e.,
was
of these
277
CROSBY
a characteristic,
too,
heating
They
were
in which,
destruction,
certainly
! This,
small,
H.
shown
theories
to
regard!ing
ie remarkably
the
pathogenesis
of the
accurate.
BIBLIOGRAPHY
Each reported
case is indicated
by an asterisk.
An asterisk
iti parentheses
indicates that
the particular
patient
had
1)een
reported
before.
Titles
without
asterisks
are research,
reviews,
historical
notes,
or cases erroneously
diagnosed
as )aroXysrnal
nocturnal
hennoglohinuria.
It is 1)elieved
that this list is complete
to January,
1950.
Asnucti’r,
I.,
Michelischeri
Kt’Hn.snAx,
F.,
Flaemoglobinurie
AND
DEN’KS,
Deutsche
H.:
Kasuistischer
Arch.
f. khins.
Beitrag
Med.
190:
zur
156.
Marchiiafava1943.*
From www.bloodjournal.org by guest on December 22, 2014. For personal use only.
278
2
P.-IJtOXYSMAL
An.n’ERnN,
j) .M,,jtN
t)
ALTS(tit’LE,
M.
cause,
L.
synudronie
I).,
ANt)
,
“Marchiafstva
tipo
S
ARNr)AL,
6
BARTA,
0. : I’ini
Scanudinav.
BIFFIS,
K.
dice)
J. : Studs’
: 1tU(1e
91,
ISO,
S.
Blood
f. laeger
M.,
m3 BRIt.E,
_,_
hull.
mm
Axm)
,
med.
et
ANt)
20
(‘.-sn
(Ic
anid
n..
erit
,
Haemoglobinurie.
Acta
1915.*
icterus
(acholuric
jaun-
1946.
of
a case
of
Marchiafava-Michehi’s
d’un
cas
(he miualadie
(Ic
\1archiafava-ImIicheli.
s#{233}rologique
med.
G.
P. : Renal
et
th#{233}rapeutique
d. hop.
function
de
(he Paris
during
65:
ha
maladie
1030,
chronic
de
1949.*
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in
man.
af
I)arOX’sfliatisk
llaenioglobinuri
niit(I.
: Cmi nouveau
#{188}1
. : La
ANI)
GAi’BE,
R. : Deux
cas (I’hi#{233}moglol)inurie
hop.
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cas (l’ani#{233}mie hi#{233}molvtique
avec
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avec
J.,
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hiSl.
t ia
di
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& Cliti.
\Led.
J.
B0UwENS
hi#{233}nuoglobimiurie
malat
ssf Marchiafavatypen.
VAX
nocturne
Paris
t)ER
et
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et h#{233}mosid#{233}ri-
is’it.h
hemolytic
1941.*
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26: 1434,
G.
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70, 1937.*
53:
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\Iilano,
.
Casa
Edit
rice
Giuseppe
l937.*
I). : Su
.
I
64,
treatment
(Ii
.
un
(‘as()
(
‘
-l’’ (‘hill . 60:
(Ii anieniiia
eriiolit ica
riniiico
21 ( E(oN
Haemoglo-
(*O)
cliniotue,
\Ian’chii:if:tv:i-\Iichieli
t il)o
paroxysmaler
.1., BF:ssrs,
\I., I’’ruvi,
.J., RM)1n.-1)UvAL,
ANt)
I)0NNET:
A POl)OS
(I’Uti
tiouvetiu
nialadie
(l(’ ‘slarchii:sf:Lv:t-)blichieli
‘
Sersiaine
d. hop.
Paris
25: 1759,
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U.,
Nt)
1)E(’on.t.E,
1.’. : Sopra
Uli
CtS()
(Ii amieniia
ennolitica
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C.Ror.1,
(‘son.o,
1946.*
paroxvsmatisk
1941.*
P.,
Soc.
-
LsBRESn
tm9
mit
F. : Haemolytic
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et m#{233}m.Soc.
938,
1luLnF:sr.Nn,
niini.
Prinicipato,
(‘:55
81:
J.
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Tilfaelde
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16 CAIN, ..,
C’n’TAx,
II..
lvtique
jet (rig#{234}mie
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tm7
nathig
1929.*
kryptogener
niurie. Presse nio(l.46: 1329, 1938,(*m3)
BtF:n.n .,A.,.
‘tNI)
\1ETTnER,
S. It : Paroxysmal
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(Marchiafava-Micheli
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ni
18
serology
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Ugesk.
med
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12
AND
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%ERNANT,
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e(I
e din.
Anaemi
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.
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haemolytica
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Oti
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Sanig2/:
10
DooD,
and
Acta
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voni
224,
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BOU5SER,
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Fall
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a BOORMAN,
102:
u. Phvsiol.
.
cons
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hemoglohinuria
68:
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f. laeger
Arch.
massive
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Ter.
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uif kronisk
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l)inurie.
[Hemolytic
:
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D.
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P. : 1’t
I.,
\A.
and
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HEMOGLOBINURIA
jaundice
an(l
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M.
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3
NOCTURNAL
,
(Id unidollo
osseo.
Rifornna
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ernol i I ica- cots
ensiosideri
.
642,
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niuria
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con
reperto
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ua
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nned
.
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192$.
22
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(I(’
‘tl:irclii:if:iy:i?
23 CnrMBoN,
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It
i qiue
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W.
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: The
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nsoct unnral
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55:
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cell
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Their
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40
41
Typ
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DAVIS
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DEnoN,
E. L.,
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Un
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ila
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or
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tipo
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59
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AND
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Utrecht
geneesk.
81:
Scansdiniav.
m’it-h
de maladie
A. : A POPO5
d’un cas
d. hop.
Paris
25: 2143,
Thesis
1935.*
HAIEN;uE,
anemia
1949.*
Ikterus
Anaemie
1934.*
L.,
N . M
,
8.80,
1938.*
I’hCmoglobiniunie.
Moscow
1505,
haemoglobinunia.
1 , 1948.*
17:
avec
ST. : Haemolytischie
13:
Nederl.
sur
KoNcniAu.0vSKAYA
med.
89 LASCH,:
report
WILKINSON,
J. Med.
Haemoglobinunie.
:
86:
U. : Chronic
Case
JACQ1’ET,
1., DROBERT,
de 1nlarchiafava-Michehi
JORt)AN,
F. L. J. : Studie
86
B.
KRMKE,
Xliii.
Quart.
87
aminenniawith
J. Med.
A. : Ict-#{232}re hCmolytique
hemoglohOnunia.
83 IsRAELS,
haemolytic
Quart-.
817, l94:3.*
28:
82 IGLA1’ER,
90
1950.
von
l9ll.*
7)
88
105,
L. K. : Chronic
MALLEY,
Marchiafava-Michehi
78 HYMANS
82
Ffllle
l943.**
77 HICKEY,
84
21:
zwei
2024, 1937.
9.5: :319, 1938.
nocturnal
1949.
(*16)
(*85)
hemoglohunsunia.]
Haeunoglobiniunie.
Wien.
nied.
Khins.
Wchnschir.
81:
193l.*
LEMIERRE
AND
Cited
KOiRtnSKI,:
by Guichsard
and
Feroldi,63
PrOt)al)b’
refers
Geux’s
to
case
H. : Contribution
nuC(l.et chin. 4: 722,
91 LEPINE,
92 LEscn-nER,
F.
335,
53
Lou,m.u
N . : Sull
,
i\-1ACKENZIE,
differemutial
190,
1935.*
J.,
r,ar
MANINu,
med.)
8: 41,
35:
Anuemia
1931.
-‘
Rev.
mens.
de
: Atypical
haemolytic
anaeniiias.
Quart
J.
Med.
8:
AND
chinico
tica
con
enuoglobiniuria-ennosicleninu
na
tipo
Niarchi
afava.
l933.*
of obscure
of
S. ANt)
CRL’K,
etiology.
Chin.
hsemnoghobinsunias.
NEVERRE,
G.
(S. I)()I)()5
(h’uns (‘aS chez
Soriderfonun
haemolytischser
Einse
.
With
notes
Misc.
Mary
Sun ha l)no(ltsdt
I ‘ensfant
ion
) . AlgCnie
Amiaemie.
oni the
I.
classification
Bassett
Flosp.
d’hCnioglobinunie
unC:I,
\Iiimsch.
rv-
5! : 354,
med.
:
l947,*
87:
Wchinsschr.
aroxysmnah
deghi
uioctunnsah
itteni
emohitici.
hennoglo-
Gior.
din.
l927.*
98 NIARCIIIAFAVA,
100
paroxystique.
U - C. Chronic
hiemolvtic
anieniia
with
Anns. Inst. Med.
23: 1)35, 1945.**
L.: Contnibuto
ansatomo-chimsico
alle studio
birsunia.
-:
l’hCtnoglobimiunie
h940.**
96 Mrt-ts’rtnt,
med.
enuohi
l3l3,
diagniosis
amsoxie
II.’
\In.’r:,
1207,
49:
G. ‘s-I. : Ilemoglobinsisnia
and
(luCe
‘anemia
med.
94. \IAt.i1EJA(’,
‘
C. U.
OSBORN,
ANt)
de
1939.
Riforma
94
0.,
a l’Ct-ude
I880.*
I.
: Aiscniia
enuolitica
cons
emosideninunia
perpetua.
Pohicli
niico
(sez.
med.)
38:
105,
109, 1928.*
emolitica
cons
emosi(lenimiunia
l)erpetua.
Pohichinico
(sez.
(*98)
A.:
(sez. med.)
NAZARI,
Nuovo
18:
241,
contnibuto
1911.
(*24)
allo
studio
(legli
itteni
cronici
cmohit-ici.
Poli-
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MARKS,
101
NOCTURNAL
Marchiafava-Michehi
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Panox’sniale
binunia). Quart.
tm02 MEYER,
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103
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104
MIc1IELI,
hamburg
105
_
(Ii Tonimso
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106
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Anemia
112
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NEBER,
J.,
ANt)
cd)nsgenital
9:
by
NEssurr,
lie
Ot,nvARn’s,
: The
use
S.,
/5:
1tm7 I’ANTON,
1,
hsaemolvtic
lichen
med.
insiproved
di med.
-emosideni
Bnit.
Sc.
M.
2574,
35:
with
ennoglohinunia
57:
tipo
emosiderinuria
90,
1931.6
mu the
1945.*
special
reference
polmonare.
to
Haemato-
Bull.
Hae-
.
PerPetila
differentiation
ons pathogenesis.
-Iar-
tipo
Marchiafava-Michieli
con
techniques
nunia
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J.
6: 137, 1947.6
cons emosiderosi
emohitica
cronica
notes
I)rat.)
1930.
Clin.
denuonistration
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ANt)
WATKINS,
F. : Et
t)E
244:3,
N.,
P.
P.,
tilfaelde
af
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Mar-
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New
England
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M.
Center
J,
circulating
antibodies
in
2: 371,
1947.
Am. J. M. Sc.
haemoglohinunia
hemolytic
anemia
74, 1942.*
203:
paroxvsmatica
(Case
nocturna.
3)
Nord.
1942.6
A.
MArTI.ANt)-JoNEs,
to
zur
ANt)
G.,
pernicious
chronsischen
ALDRICtI,
C.
ANI)
Arch.
f. kIm.
A. : Chronic
(I’larchiafava-Michiehi
A. : I)e
ziekte
old
van
G. : The
RtDD0CII,
anaemia.
Lancet
h#{228}moI’tischen
Deutsche
thsromhocvtopensia
mi a five-year
lioninNsoN,
J. H. : tniniary
excretion
RonHARt,
of
albumin
medium.
Blood
C. H. : Acute
porphyria.
H#{228}nuglobirsunie.
PIERcE,
m18,.
1946*
Accad.
(sez.
erythnocytes,
Roma
W. : Newer
anemia;
nail hemoglobinunia
11)
anemia
disorders
A. : Beitrag
117,. PETERsEN,
It)
cons
Accad.
1)AMESHEK,
B.
med.
381,
1947.
-
the
ins
emolitica
hennolytic
166,
ANI)
114
35:
l)erPetU2t.
emoglobinunia
transsfused
anuaemia.
(Ii anemia
l946.*
caso
di
roy.
54,
cons
of
Uns caso
:385,
terzo
A. : Un
haemoglo-
Haemoglohinunie.
Pohichinico
101 , 1931 (*98)
haemnoglobinunia.
survival
G. : Sopra
(hiafava).
113
12:
of nocturnal
19: 353, 11)38.
, M . : Arseniia
NAZARI,
4:
Sang
haennolytic
29:
paroxysnnaler
enuosiderinunia
perpet-ua.
emohitica
L. : The
matologica
Praxis
von
Form
con
ennosideninsunia
hCrnolytiques.
of acquired
logica
MORTAR
Marchiafava.
Typus
nieueni
ernohitica
IIaeniatologica
P.
cases
110 MONTALt)O,
I
con
J. A. : A case
109 \1ottisoN
nocturnal
1928,(*98)
(splenomegahia)
chiafava.
108 Mun.NE,
In
di anemia
caso
107
zmi einer
(paroxysmal
i9:3.
F. : Un
1928.
: Spl#{233}nsonnCgalies
syndrome
18: 105, 1949.***
H#{228}moglohinunie
Beitrage
Thesis,
HEMOGLOBINURIA
Med.
196:
hemolytic
anemia
syndrome)
.
child.
J.
of carbonic
Nederl.
486,
of
some
msacht-
1949.6
with
Report
Pediat.
22:
msnshy(lrase.
Marchiafava.
relationship
1: 529, 1924.**
An#{228}mie mit l)arox\’smaler
paroxysmal
of a case
30, 1943.6
J. Chins.
tijdschr.
v.
noctur-
with
Path.
3:
geneesk.
marked
142,
1950.
90:
1866,
1946.6
i19:
.
(‘hirotsiso-hie
hiaenuolvt
mlar(-hiiafava-\hidhieli
m20 ROSENTHAL,
I”. : then
kIm.
Med.
Bo’nin,
0.:
Med.
110:
122 11 USCICA,
123
-:
ansaenuie
des
“IctCre
77, 1913.6
G.:
Enuosideniniunia
124 SALAH,
[sic]
n25 SALN,
66:
SAXL,
chir.
38:
M.,
AND
syndrome).
E. B.:
566,
P.:
792,
niet
haennoglobiniunie.
(Ziekte
Hiimoglobinurie.
vans
Ztschr.
h#{233}molysinique”
in
ansemia
prat.)
con
(Chiauffard).
(splensomegahia)
38:
Deutsche
Arch.
f.
f. kIm.
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1509, 1931.6
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Arch.
1932(6122)
GInANEM,
M. I).:
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Report
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J. Roy
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1927,*
Discussion.
nacht-ehijke
1950.
(*119)
paroxysmaler
1932.6
(Nota
preliminsare).
Polichinico
(sez.
Anemia
(splensome:.alia)
emolitica
ital.
126
119: 449,
Zur Frage
ische
I . .Annsterdann
Thesis,
einse
neue
Form
voni
Wieni.
med.
Wchnschsr.
haemoglohinunia
Egyptian
M.
paroxysmalis.”
78:
553,
1928.*
(Marchiafava-Michsehhi
A. 32:
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Scandinav.
Soc.
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WILLIAM
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128
248,
128*
128b
inies
i)ij
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een
VAN
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J.
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f.
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86:
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Mededeehinigeni
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door
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7:
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ed
emosiderinuria
508,
55:
anatomo-patologico
Marchiafava-
treated
with
sulphathiazole.
1949.*
emohitica
prat.)
tipo
1938.*
haemoglobinuria
& Hyg.
Pohichinsico
82,
recurrent
Med.
E. : Un
D.,
WASSERMAN,
Am.
con
1302,
emoglobinuria-emosiderinuria
tipo
1948.6
dell’ittero
ternuituating
STnrnn,
Axn
Pat-h.
18:
of
a
emolitico.
ANI)
Policlinico
: ther
Splenomegahie.
(sez.
med.)
P. A. N.,
heniohitica
tipo
khins.
22:
con
C.
Marchiafava-Michseli.
N(; , 1. : Paroxysmale
138 STRtBn
139 TRESTmNI,
S. : Ln
VAN
AND
globinsunie
I #{149}(
‘RI:
Thesis,
141
,
,
DEN
l.
setneiologico
M.,
d’ansemie
hCmolytioue
P.
hsro-ytcs
F.,
ADAMS,
in
M.,
5:
1643,
223
and
(Case
1938.6
231,
1880.
Icterus
0.
hemoglobinuria
mit
2)
G. : Anemia
paroxistica
Santa
Fe, Argentina
9: 5, 1942.6
I)cutschse
me(i. Wchrsschr.
8: 1 1811(117, 1882.6
nsue(l.
tipo
(he Marchiafava--Iichehi
mt
by
)bloscow
19:
(‘omit m’azionse
Soc.
med.
J. F.: The
cdl emosideriniuria
19: 235,
11)39.*
i ni de hiaemiiolv
(-hsan’actenize(l
mmcl urnal
urethra
Hamoglobinurie
ANm) B0NAzZoLA,
y
A.
et
43,
(henio-
t ischse
della
hiemnolvsis
niilza,
SmKoutAv
AND
d. liSp. de Paris
iron
metabolism
J.
une(hianite
II.:
Sun
(syndrome
55:
49:3,
an(1 acid
Clin
ansd(’n
.
hiCmoglobinsunie
hsemoglobinsuu’ia.
t oest
ravascular
1941
volunie
di
l92S.
Ii., RUBENS-DUVAL
et mCm.
ANI) Ross,
aroxysmal
A.,
hiCmosidCninsunie
avec
Bull.
H.
(he rued,
trop.
ichse
fact on(’n
(helLs
465,
JUSTIN-BESAN#{231}ON.
\Iarchsiafava-Michschi).
\%TA(;Lt’7Y,
12:
e cinc.
cases.)
the
acholurischen
cons emoglobinsunia
.
hemo-
1794.
n#{228}chthicher
45:
42: 551), 1935.*
L. : I.e svndronue
,[ Elms
from
1: 332,
perrnanente
aniemiiia
syndrome)
Cuore
1906;
Soc.
belge
(her h)ltSnsitt
19.14,6
F. ?mI. : [Chroniic
: Ii sigmiificato
adrensahinsa.
143 VIu.LARET,
med.)
BERCtIE,
Aniri.
J . : St udie
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emolitica
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nocturne).
N t)E N IIR()
Leuveni
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med.
med.
6 other
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1949.6
Wchmsschr.
chronischens
BUSANICHE,
Rev.
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Policlinico
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140 \ALCKE,
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H.,
of
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H.
STRA(#{149}SS
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757, l948.*
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W.
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e din.
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1915.(*8)
134 STATS,
142
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emohitica
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389,
‘--
Norsk
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\‘A
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I I O.
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136.
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l938.**
130 SEGA,
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1944.6
: Over
529 SCOTT,
133
der
697,
127:
BEI’RDEN,
ann
(;unstige
uitwerking
hiuls 1: 15, 1944. (*128)
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SCHOONHOVEN
AN
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283
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146
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ins parox’snial
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1951 6: 270-284
Historical Review: Paroxysmal Nocturnal Hemoglobinuria: A Classic
Description by Paul Strübing in 1882, and a Bibliography of the Disease
WILLIAM H. CROSBY, LT. CoL.
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