From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
“Hairy”
Cells
in
Blood
Disease
in
and
“Flagellated”
Normal
By
T
HE
FIRST
ROBERT
lymph
Studies
this
massive
cells
cells.”
These
cells”
described
The
that
cells
second
studies
and
definite
to he
Mitiis
object
lymph
large,
seem
paper
is to
had
the
report
suspensions
and
The
flagellated
elongated,
thin,
the
flagellated
blood
of the
observed
peripheral
arbitrarily
cells
microscopic
normal
were
and
few
projections.
of
patients
“hairy
reticulum
contrast
of
cytoplasmic
two
called
of 5 patients.
phase
cells
marrow.
showed
lymphoid
blood
to
in the
disease
palpable
“neoplastic
peripheral
is
spleens.
no
were
in
between
cells
and
to the
in the
paper
or
cells
numerous
hairy
villi
identical
the
a rare
few
“flagellated”
nodes
similarities
report
cells in the blood
and bone
phase
contrast
microscopy
short
et al.1
of
so-called
on
plastic
numerous
by
J. DONNELLY
WILLIAM
splenomegaly,
had
of
Nodes
nodes,
and abnormal
mononuclear
of the
viable
blood
cells
with
peculiar
and
of
with
AND
Neoplastic
Cells
Lymph
SCHBEK
OBJECT
in 2 patients
Lymphoreticular
lymph
number,
There
nodes
of this
hyper-
in
were
and
spleen
report.
METHODS
Special
studies
on
on all patients
who
Hematology
Section
Hines.
Illinois,
and
are made
on leukocytes
The methods
used
kocytes
of 20 ml. of
leukocytes
are performed
in tile
Tumor
Research
Laboratory
found
to have
lymphocytic
leukemia
or lymphocytosis
in the
of the
Medical
Service
of the Veterans
Administration
Hospital,
on some
patients
from
other
hospitals.
In addition,
similar
studies
from nonleukemic
patients
with normal
hemograms.
have
i)een
described
previously
in detail.2
In
brief,
the blood
leuheparinized
blood
are concentrated
by sedimentation
and are washed
and
equal
resuspended
(Grand
in
Island
viable
are
parts
From
the
First
Tumor
Medical
by grant
submitted
ROBERT
Services),
fresh
Northwestern
Hematology
ciate
Chicago,
Research
Grand
human
serum
and
Fischer’s
medium
No.
147C
23,
M.D.:
Island.
Laboratory,
Service,
Veterans
CA-07541
From
Feb.
SCHREK,
Veterans
Co.,
of
N. Y.).
A drop
of the suspension
is placed
in a special
slide
chamber
composed
of two large
cover
slips separated
by a metal
plate
(1 mm.
thick)
with
a central
hole.
The cells sediment
on the lower
cover
slip and are
examined
with an inverted
phase
contrast
microscope.
The leukocyte
suspensions
are also
incubated
with
and without
reagents
in small
test tubes.
At periodic
intervals,
usually
once or twice
a week,
the incubated
suspensions
are transferred
to slide
chambers
and
the viable
cells are studied
and
counted
in an area
measuring
10 x 0.04
mm.
Section,
Aided
Biological
1965;
the
accepted
Chief,
Administration
Research
Department
of
National
for
Cancer
Tumor
Research
Hines,
Stritch
Services;
limes,
USPHS,
July
24,
Laboratory
Ill.;
Ill.
Sc/moo!
of
BLoon,
VOL.
Bethesda,
and
Professor
J.
Administration
hematology
Md.
1965.
(Surgical
Associate
\VILLIAM
and
Illinois.
Institute,
publication
Hospital,
Medicine,
Surgical
IIo.sital,
University
Medical
Sc/moo!, Chicago,
Section
(Medical
Service),
Veterans
Professor,
and
Administration
of
DONNELLY,
Hospital,
Medicine
of
Research
Pathology,
M.D.:
Hines,
Loyola
Chief,
ill.; A.csoUniversity,
Ill.
199
27,
No.
2
(FEBRUARY),
1966
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
200
SCHREK
AND
DONNELLY
OBSERVATIONS
Patient
A
on
W
68-year-old
the
Negro
13,
August
6 months
the
not
of
was
15-17).
were
ly,
Veterans
and
loss
the
except
abdomen
and
10.0
Cm./100
ml.
mm.
(direct
method).
of
a
thin
blood
and
film
The
cells
present.
apparent
were
cytoplasmic
and
Administration
of
15
pounds
for
a massively
extended
Hospital
of
weight
(luring
enlarged
below
Leukocyte
count
An
a
spleen
the
was
iliac
crest.
7300/cu.
mm.
( Coombs)
antiglobulin
May-Cruenwald
differential
round
borders
pseudopods
or
were
were
ovoid
test
shape,
indistinct
present.
The
Ciemsa
count
showed
of
8
poly-
and 79 abnormal
mononuclear
size of normal
lymphocytes
(see
in
often
and
leukocyte
forms,
10 lymphocytes,
one to two times
the
generally
Cytoplasmic
with
stained
polychromasia
morphonuclear
neutrophils,
3 band
cells. The mononuclear
cells were
Figs.
Hines
present.
120,000/cu.
anisocytosis
forms
the
pain
results.
Examination
moderate
to
abnormalities
side
concentration
numbered
negative
admitted
periumbilical
significant
left
was
Hemoglobin
gave
no
entire
Lymphadenopathy
Platelets
of
admission.
disclosed
occupied
was
because
before
Examination
that
man
1963,
although
and
a few
slighfly
cytoplasm
elongated
serrated.
was
pale
Rare-
blue
in
color
The
cytoplasm
of a few
cells
contained
1 to
10 small
azurophilic
granules.
The nuclei
were generally
round
or oval in shape,
occupied
from 50 to 80 per
cent of the cell, and were either
centrally
or eccentrically
located.
The nuclear
membrane
was distinct.
Nuclear
chromatin
was moderately
fine and had a spongy
appearance.
In a
and
homogeneous.
few
cells one or more faintly
visible
nucleoli
were present.
Bone
marrow
particles
obtained
by sternal
aspiration
were
and contained
slightly
decreased
megakaryocytes.
Approximnately
were
normoblasts.
The
and
A
remainder
were
more irregularly
very
few
plasma
cells
cells
Fig.
similar
18).
mononuclear
shaped
(see
and
moderately
25
granulocytic
to those
hypocellular
per
precursors
in the
blood,
but
cent
of
were
present.
frequently
cells
larger
A tentative
diagnosis
of “atypical
leukemic
lymphosarconia”
was made
and a course
of roentgen-ray
therapy
to the spleen
was given.
After
administration
of 548 rads in 18
days with a field of 15 x 20 cm. to 12 x 20 cm., the splenic
size had not changed.
but
the leukocyte
count
had fallen to 460/cu.
mm. and the hemoglobin
level to 6.8 Cnl./100
ml. Four
units
of whole
blood
were
given
and
the
patient
was
dismissed
from
the
hospital.
One
month
size.
Three
in
following
months
subsequently
later
remained
Cm./100
ml.
leukocyte
counts
radiotherapy,
only 5.0 cm.
Hemoglobin
counts
the
spleen
below
levels
the
have
had
left
have
except
decreased
costal
ranged
have
ranged
from
1100
to 2800/cu.
never
revealed
more than a few of the abnormnal
ranged
from
74,000
to 106,000
(direct
method).
for vague
abdominal
distress
when
last seen on
have
asymptomatic
of
felt
unchanged.
Leukocyte
counts
Platelet
completion
it was
slightly
has
margin
from
and
10.4
to
12.6
mm.
Differential
mononuclear
cells.
The patient
was
May
26, 1965,
21
months
after diagnosis.
Blood
was obtained
for study
of viable
leukocytes
on August
15 and 19, 1963, before
the patient
was irradiated.
A suspension
of the blood
leukocytes
was prepared
in 50 per
cent normal
human
serum
and the cells were
examined
in a slide
chamber
with
an inverted
phase
contrast
microscope.
In the suspension,
86 per cent of the nucleated
cells
were
about
angles
to
8
the
in
diameter
circumference
of
and
had
the
cell
numerous
(Figs.
thin
1 and
2).
cytoplasmic
The
projections
projections
varied
at
in
right
length
but were
usually
short,
about
1-2
in length.
The nuclei
were
usually
large,
oval with
a few soft chromatin
masses
and occasional
small
nucleoli.
The cytoplasm
was slight
in
amount
with a few mitochondria.
Some cells had a large
(3-5
oval clear
mass in the
cytoplasm
(Figs.
3 and 4).
The
cytoplasmic
mass
was characterized
by a few linear
striations
or small
amorphous
gray
material.
A few
cells
had
2 or 3 of these
masses
(Fig.
5). The cytoplasmic
mass compressed
the nucleus
which
was crescentic
and next to the
cell
wall.
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
HAIRY
CELLS
IN
201
BLOOD
:1
G
1
2
I
,.
:
I1p$p.’J
4
6
Figures
1-14
are photomicrographs
of viable
graphed
with an inverted
phase contrast
microscope,
Figures
1 to 7 show
“hairy”
cells from patient
Figs.
1 and
from tile entire
tin masses
and
Fig. 3.-Hair
with
gray
smooth
with
Fig.
masses.
4.-Two
soft
gray
5.-Hairy
cell
(fromn
circumference
nucleoli.
cell
(from
cell
The
masses.
Fig.
2.-Hair
patient
of cells.
The
W)
nuclei
cells
in slide
magnification
W. and figure
with
mllmmerous
are
large
with
chambers,
photo2000 x.
8 from patient
Y.
short
villi
light
extending
gray
chroma-
iNitiemit
W)
with
numerous
fine villi and lymphocyte
wall.
The hairy
cell has a large
clear
oval vacuole
with
small,
soft
nucleus
of tile hair
cell is crescentic
in shape.
hair
cells
(from
pttient
\V),
one with
large
round
clear
vacuole
masses
and
cell
(from
linear
striations.
patient
\V)
with
3 large
vacuoles
with
small
Fig. 6.-Two
hairs’ cells
(from
pttiemit
\V)
incubated
I clay.
The nuclei
crescemitic
and anteriorly
plicecl.
The cytoplasm
is increased
in amount
finely
granular.
One cell has a few anteriorly
placed
cvtoplasmic
projections.
Fig. 7.-Hair
cell
(from
patient
\V)
irradiated
with
1000 r and incubated
37 C. for 8 days.
gray
are
and
at
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
202
SCHREK
The
cells
viable
hairy
were
cells
Bouroncle
et
et
al.1
al.3
The
in
The
small
hairy
by
by
According
the
that
formnation
of normal
6).
cell
cells
To
test
An
for
with
1000
days
of
these
the
in
1964,
Y
A 46-year-old
pital,
Chicago,
the
The
hairy
were
were
normal
to
Three
was
on June
and
the
lymphocytes,
masses
differentiated
in
prQjectons
usually
the
milovedi
associated
with
was approximately
as
showed
no mnotility.
not
in
shape
became
have
or
in
as
crescentic
were
fewer
in
cells had a few
macrophages
seen
fast
elongated
smaller,
projections
of some
were
the
suspension
as
the
preparations
abnormal
present
to
are
seen
suspension
in-
with
1000
of
of
patient
\V’s
ml.
neutrophils,
(Fig.
19).
bone
surgically
cells
granulocytic
(Fig.
irradiation
and
and
each
of
of the cells in
17 to 28 per
those
as
seen
in
the
described
pre-
Veterans
Administration
in the left lower
part
Hosof the
a
palpable
liver
left
costa!
margin.
leukocyte
were
of
cm.
below
was
4200/
10 mnonocytes,
48,000/cu.
mm.
unsuccessful.
sternal
hypoplasia
20) which,
on
and hone marrow.
9
count
55 lymphocytes,
Platelets
numbered
portion
15
On
Only
masses
1000
January
Most
to
during
7
in 1 day.
with
irradiation.
similar
The
marrow
excised
blood
cells
cells
lymphocytes.
Cm./100
megakaryocytes,
mnononuciear
in
a
hairy
hairy
and
the
irradiation
of the
in number.
below
7,
all the
few
except
10 cm.
cell
Figure
1963,
abnormalities
35
irradiated
and
on October
30,
leukopenia
following
to the \Vest
Side
because
of pain
spleen
was
by
admitted
29, 1964,
no
the
1
killed
were
all
hairy
attempts
numbers
those
see
nucleus,
chromatin
cytoplasmic
nuclei
cells
neutrophils
aspirate
identical
easily
irregular
the
were
but
hairy
the
concentration
method).
by
be
cells which
were
cells had cytoplasmic
disclosed
margin
seen
were
of
man
again
had
(direct
adequate
glass
all the
phagocytized
normal
small
motility,
did
hairy
suspension
10.0
touch
the
to
were
of
with
became
cells,
was
filtration
number
however,
and
few
was
vealed
suspension
villi or cytoplasmic
7 days,
the cytoplasm
patient
cells
A few
hemoglobin
and!
on
the
movement
cells
the
cii. mm. Differential
leukocyte
count
and an occasional
abnormal
mononuclear
tions
any
nearly
difficult
the
cells,
amount
studied
was
the
cells
white
Illinois,
costa!
flattened
In contrast,
or eccentric
slight
however,
in the
when
Examination
right
Mittis
avoid
frequently
could
The
A
of the
mononuclear
Patient
to
a central
nucleus
studies,
suspension,
hairy
suspension.
abdomen.
was
small
showed
The
for
monocytes.
lymphocytes
original
viously.
paper
with
to
oval
of the hairy
days,
suspensions
the
by
by
ectoplasm.
lymphocytes
more
of the patient
leukocyte
of
it
for 1 to 7 days.
As seen
in Table
r had little
or no effect on the survival
but irradiation
with 8000 r killed nearly
examinations,
cent
the
described
at 37 C.
2000
18,
this
cells.
incubated
the
respects
as
described
suspension
that
a large
cell
Most
radiosensitivity
blood
The
the
increased
The
days
to
November
so
of
and
placed.
incubation
normal
incubation,
In contrast,
r in 7 days.
in
of the
flattened
pseudopods.
1 or
granules.
and
r
ieukocyte
cinemicrographic
cytoplasm
21
many
cells
cells
retained
thinness
ring
occasional
of large
hyaline
lymphocytes.
from
In
leukocytes.
motion
the
8000
the
slightly
wall
and
in shape
and
eccentrically
number
but longer.
After
to
be
thick,
were
and a thin
fast
The
coarse dark
to develop
cubated
for
in
were
cells
iregularly.
incubation
After
cells.”
histiocytic
reticulum”
however,
extreme
but
hairy
hairy
to
and
will,
and
flattened
of viable
slowly
(Fig.
size
a smooth
The
(Fig.
3).
suspensions
“hairy
or
(1 and 2
in diameter)
added
but not by the hairy cells.
to the hairy
cells, there
were
a
addition
characterized
of
reticulum
lymphoid
granulocytes
of endoplasm
particles
name
the
DONNELLY
classification.
large
not
descriptive
cells”
to
of
the
granulocytes
In
“neoplastic
A few
zone
Latex
the
to
“hairy
were
clearly.
a wide
to
regard
by
cells
nuclei
similar
number
indicated
as
the
were
and
term
commitment
given
AND
review,
These
Microsec-
bone
and
were
cells
marrow
a
re-
diffuse
seen
were
imi-
to
initially
be
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
“HAIRY”
CELLS
Table
1.-E
203
IN BLOOD
ffect
of X-Irradiation
on the Survival
of Hairy
in a Leukocyte
Suspension
Prepared
from
Bklod
August
19, 1963
from
Patient
W
Viable
Days
0
Doseof
irradiation
(r)
of
-
Incubation
at
37
C.
4
-
7
-_________
Lymphocytes
Hairy
cells
Lymphocytes
Hairy
cells
Lymphocytes
Hairy
cells
344
845
154
649
51
8000
48
0
17
0
4000
546
26
242
6
2000
956
125
376
14
1000
970
117
0
1160
consi(lered
A
diagnosis
of
July
performed
tiomls showed
was
immature
lymphocytes,
reticulumll
cells
26
279
0
245
0
and
primitive
marrow.
The
October
14,
platelet
mm.
by
abnormal
patient
on
made
returned
and
the
patient
treated
with
pre(lflisOfle
ieukocyte
cells
4,
14,
1964.
2
According
in
and
leukocyte
a slight
bone
hypoplasia
found
The
for
marrow
a striking
the bone
count
increase
in
aspiration
infiltration
marrow
and
spleen.
diagnosis.
of this
1964,
mlll.
except
A successful
previously
after
Gm./100
normnai
granulocytic
11 months
blood
10.0
was
mm.
and
cells
October
November
was
count
280,000/cu.
erythroid
viable
on
normal.
level
mononuclear
the
to
hemoglobin
is asymptonlatic,
microscopy
was
of spleen
and liver
becaimse
of persistent
numbered
showed
Unfortunately,
studied
the
Differential
Platelets
same
size
count
1965,
1965,
the
lymphoma
decreased
slightly.
Spienectomimy
was
14, 1964,
thrombocytopenia
and a desire
to
for diagnostic
study.
The
spleen
weighed
750 Gm. Touch
preparainfiltration
of the spleen
by cells (Fig.
21) identical
to those in the
tissue
lymphocytes.
on April
14,
trast
and
The
27.
a diffuse
5150/cu.
The
“mature
mnaiignant
18 to July
on August
additional
obtain
On
of
Lymphocytes
lymphocytes.”
from
bone
185
a mixture
atypical
Lymphocytes
cells/mm.3
1
----________
Hairy
cells
Cells
and
Obta med
patient
months
to
were first examined
by phase
conafter
splenectomy
and
were
again
cell counts
of leukocyte
suspensions,
viable
3 to 5 per cent of the cells were hairy
cells. The
hairy
cells had many
short
cytoplasmic
villi. The nuclei
were
fairly
large
and somewhat
lighter
than
nuclei
of lymphocytes.
Cytoplasm
was slight to moderate
in amount
but (lidi
not have
the masses
seen in the first case. The hairy
cells were
similar
to those
seen in
there
the
was
a relative
first
became
smaller,
affected
by
for
irradiation
hairy
cells
human
In
(8-10
some
of
)
cytoplasmilic
similar
the
Nodes
in some
nodes
suspensions
cells
(Figs.
were
The
tile nuclei
crescentic
respect
cytoplasm
cell.
was
The
cells
usually
were
moderate
resistant
(Fig.
in
6).
amount
The
and
hairy
to cells
observed
previously4
for
diagnostic
purposes.
removed
normal
lymph
the
cells
nuclei
were
not
nodes,
Fast
a
small
by
milOtion
without
to see
in suspensions
On
histologic
or hyperplastic.
characterized
9-14).
were
large
and round
in shape
or branched
increased
\V
case
Spleen
considered
from
These
and
were
were
in
8).
r (Fig.
which
cytoplasm
as
projections
waved
haphazardly
nuclei
of the cells were difficult
cells,
were
the
lymph
seen.
tion.
of
1000
nodes
projections
cytoplasmic
The
were
the
were
days,
eccentric
of Lymph
lymph
the
more
and
with
Cells
examination
1 or
crescentic
“Flagellated”
The
and
patient.
incubation
After
from
lymphocytosis
number
numerous
cinenlicrography
any
and
perceptible
were variable
of
large,
large
tilin,
round
cells
flagella-like
showed
that
the
rhythm
or synchronizain maturity.
In a few
with
nucleoli
(Fig.
13). In other
cells, the nuclei
and were
eccentrically
placed
(Figs.
12 and
14).
in amount
and appeared
dark
due to the thickness
to irradiation
with
1000
r and
survived
1 or more
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
‘
t,1’
#
p
‘S
.4.
P
‘
Si”
..
‘1
‘V
a,,
s
114
S
13
Fig.
8.-Hairs’
cell
(from
patient
Y)
irradiated
with
1000
r amid incubated
for
5 days.
Figures
Fig.
9 to 14 are “flagellated”
9.-Cell
with
numerous
from
entire
circumference
structure
with
a nucleolus.
plasia,
patient
cells
from
elongated
of cell.
Nucleus
(l)iagmiosis
of
lymph
thin
can
l’mph
nodes.
ctoplasmic
projectiomis
be vagueh
made
out
node-reticuloendothehal
as
extending
an oval
hvper-
L.)
Fig.
10.-Cell
with
radiating
cytoplasmic
projections.
(Diagnosis
of l’smph
node
-dermatopathic
lvmphodenatis.
Patient
J.)
Fig.
11.-Large
flagellated
cell with
numerous
ctoplasmic
projections
amid smnall
lymphocyte,
slightly
out of focus.
The
cytoplasm
of flagellated
cell is finel’
granular. (Diagnosis
of lymph
node-dermatopathic
i’mphodenatis.
Patient
B.)
Fig.
12.-Two
large
flagellated
lmranching.
(Diagnosis-chronic
Fig. 13.-Flagellated
cell
is small
with
a small
Fig.
14.-Cell
clear,
amid crescentic
node-no
specific
nucleolus.
cells.
The
lvmphodenatis.
with numerous
nuclei
(Diagnosis-no
specific
with
numerous
short
ctoplasmic
in shape
amid is just beneath
lesion.
Patient
P.)
204
are
thimi,
Patiemit S.)
short cvtoplasmnic
the
lesion.
projections.
cell wall.
clear,
elongated
projectiomms.
Patient
P.)
The
nucleus
(Diagmiosis
and
Nucleus
is thin,
of lymph
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
“HAIRY”
row
CELLS
Figures
films
Figs.
205
IN BLOOD
15-17.-Abnormal
nuclear
chromatin
‘isible
in Figures
15
18.-Bone
marrow
\V. One normoblast
spongy
fainth’
Fig.
patient
those
of stained
15 to 21 are photomicrographs
and in touch
preparations.
1000
in the
mononuclear
and
poorl’
cells
in blood
and
bone
mar-
of patient
W.
borders.
Nucleoli
Note
are
X
cells
defined
in blood
cvtoplasmriic
film
amid 16.
film
and
macic
cluster
from
particles
abnormal
of
aspirated
from
sternum
cells
similar
mononuclear
of
to
blood.
days
after
irradiation.
These
cells,
covered
with
numerous
elongated
cytoplasmic
projections,
were
called
“flagellated
cells.”
This name
is merely
descriptive
and
does
not
imply
anything
in regard
to the nature
or functions
of the cytuplasmic
projections.
In a few hours
after
incubation,
a few
lymphocytes
became
a(llilrent
to the flagellated
cells,
which
usually
became
covered
with
so many
lymphocytes
that
it was
frequently
difficult
to see the centrally
placed
flagellated
cell.
However,
tue long cytoplasmic
projections
sommietimes
extended
beyond
the cell mass
and sonic
flagellated
cells were
near
the edge of the cell cluster
and
could
be clearly
seen. The adhering
lymphocytes
remained
viable
and in good
condition
for many
days.
DIsCusSIoN
A
Pectmli1r
type
suspensions
of
descriptive
name
In
stained
mistaken
cell,
for
elongated
had
irradiation
normal
The
sarcoma
with
1000
or
elongated
microscopy
contrast
The
in
was
given
the
resembled
and
was
cell
Some
numerous
of
characteristics
of
and
the
The
masses.
shape
cell
hairy
lymphocyte.
had
radioresistant
These
the
malignant
cytoplasmic
highly
r.
Phase
pttiemits.
films
projections.
was
2
lymphocyte,
the
than
cell
from
marrow
atypical,
normal
1 to 3 large
rather
hairy
cells
hone
a normal,
the
by
observed
was
cell.”
and
cytoplasmic
patients
The
cell
blood
of “hairy
blood
unlike
shape
of
viable
the
hairy
cell
normal
was
The
short
viable
cells
of
assumed
a
few
of
the
irregular
lymphocyte.
killed
differentiated
one
an
amehoid
not
hairy
or
villi
in
hairy
one
week
cells
by
from
lymphocytes.
lymphocytes
from
some
patients
with
chromiic
cell leukemia
have
some
of the characteristics
lymphocytic
of hairy
or
cells,
lymphonamely,
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
206
SCHREK
AND
DONNELLY
19
ES-.
C
21
Fig.
19.-Abnormal
Fig.
marrow
Fig.
20.-Touch
of patient
21.-Touch
preparation
cells
similar
a few
motility,5
flagella-like
prolonged
However,
it
lymphocytes
to flatten
hairy
cells
in blood
film
surgically
of spleen
to those
in blood
cytoplasmic
survival
was
by
contrast
cell
of
excised
of pittient
Y.
specimen
of
sternal
bone
Y.
miuclear
In
mononuclear
preparation
easy
the
to normal
themselves
did not
to
numerous
of patient
Y. Predominant
bone
marrow
of both
amid
projections,2
time,6
and,
differentiate
irregularity
a few
in
the
short
villi
monocytes,
and
hairy
hairy
the
cells
on glass
and
did not
develop
into macrophages
cells
patients
in
cases,
cells
and
the
phagocytize
as occurs
a limited
latex
with
nuclei.
tendency
particles.
normal
The
mono-
cytes.
In conclusion,
but
neither
lymphocytes
hairy
is it possible
or monocytes,
cells
cannot
to exclude
or with
be
classified
a relationship
both.
as lymphocytes
of hairy
Y.
leukemic
of the
only
monoaudi
shape,
reduced
radioresistance.7
characteristics
had
are
\V
or monocytes,
cells
with
either
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
“hAIRY’
CELLS
The
clinical
similar.
Both
leukocyte
were
findings
had
counts
found
in
spleen
of patient
In one
patient,
number
hairy
literature
cells
identical
by
other
the
blood.
for
Second,
clinical
Mitus
et
examined
al.1
in
stated
the
nized
living
of “abnormal”
except
that
and
are
cells”
of
to
of
see
a
determine
described
the
small
whether
and
whether
marrow,
classified
others
have
massive
and
a benign
course
differential
5 to
cent
18 per
cell
is
or
histiocyte
the
clinical
diagnosis
serrated
edges
count.
Most
moderately
observed
some
initial
of
the
either
in
and
in
leukemic
viable
of the
leukopenia
some
of
Bouroncle
had
et
its
al.3
on
fine
by
patients
were
acute
or
diagnosed
chronic
James
the
thrombolymphocytosis.
stained
blood
fibrillary
films
projections
to
blood
and
nontender
spleen
resulted
in
in all 3 patients.
reticuloendotheliosis.
reticulum
patients
lymph-
moderate
characteristics
in
recog-
especially
firm,
relative
done
that
Radiotherapy
in prolonged
given
large,
2 patients,
counts
cells
authors
not
of their
“Neoplastic
anemia,
in
The
type,
was
with
normochromic
from
cells.
cells
patients
with
varied
frequently
the longevity
cell sarcoma.
were
borders
cells.
are
to hairy
cells
counts
of lymphocytic
hairy
3
irregular
reticulum
cells
and
The
of
the
26
These
of
a good
reticumlum
patients
authors
with
stress
cells.
count
in 25 of the 26 patients
of Bouroncle
mm.
with
0 to 95 per cent
reticulum
cells
as
or
have
cent
identical
leukocyte
by
of the
per
resembling
blood.
Leukocyte
not report
reticulum
reticulum
of
total
leukocyte
to 23,000/cu.
laboratories
did
with
count,
similar
seen
to
to be
described
borders.
patient
and
cell
seen
motile.
90
splenomegaly
peripheral
reticulum
normocytic,
3 patients
hairy
were
probably
leukocyte
their
cytoplasmic
response
in one
The
They
died
prominent
the
considered
atmthors
normal
with
not
6 to
of cells
moderate
all
marked
a hyperspienic
2 patients-namely,
bone
in
lymphoid
are
These
cytopenia,
and
were
usually
description
spleens,
patients,
cells
in
and
peripheral
lymph
nodes,
normal
relative
increase
of “lymphocytes”
with
and
the
total
splenectomy
persistence
first,
to
by
patients
with
variety.
patient
one
Goodwin.s
showed
state
neoplastic
reticulum
A good
was
was
The
thrombocytope-
and
been
were
mononuclear
both
patients
other,
but
have
of
cells
hut
mm.,
the
such
as
5
reticulum”
18,200/cu.
that
1000
necessary
in blood
described
extrusions
to
3200
The
was
enlargement
counts
cells
patient
purposes:
cells
presented
or no
leukocyte
lymphoid
pseudopodic
the
syndrome
two
hairy
it
splenomegaly.
in the
symptoms
blood
disease.
“neoplastic
In
in the
the
in
a megakaryocytic
one
of
searched
mononuclear
of the
of
cells
little
elevated
or other
therapy
to the
to
hypersplenic;
alleviation
or similar
splenomegaly,
or slightly
had
clearly
was
authors.
observed
oid
by
related
cells
were
normal.
Abnormal
the bone
marrows
of
patients
was
hairy
with
primarily
X-ray
followed
of
The
2 patients
Y. Both
this
likely.
is
was
the
symptoms
nia.
mechanism
of
of both
patients
large
numbers
in
the
other
207
IN BLOOD
originally
lymphocytic
in
other
leukemia.
in
et al. was
the initial
hematologic
The
spleen
markedly
enlarged
in 25 patients
and
lymphadenopathy
only
9 patients.
The
clinical
course
was
chronic
in
of whom
splenomegaly
had
a predominantly
and
minimal
“splenic
invasion
form”
of peripheral
of
12
disease
with
blood,
bone
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
208
SCHREK
marrow
and
responded
patients
favorably
gave
good
after
lymph
nodes.
These
et
theliosis
al.#{176}
reported
with
their
3
were
nodes,
cells
frequently
49
lymphoid
patients
lymph
reticular
cells.”
patients
with
splenomegalv
Splenectomy
died
3, 7,
in 5 chronic
and
16 ‘ears
Gosselin
et al.#{176}
stressed
diagnosed
“leukemic
authors
frequent
whom
findings,
5 patients
Four
had
a patient
for
many
considered
to
leukemia.
In this
extended
apparent
below
the
iliac
lymphoproliferative
and
warrant
patient
a search
The
hairy
cells
seen
in suspensions
covered
of the
istics
nucleus.
the
of
between
imprints
cells”
cytoplasmic
adjacent
with
of
adhering
lymphocytes
blood
differential
its
lymph
to
nodes.4
can
leukocytes
has,
viable
be
seen
however,
a
of
only
the
of
the
cure
lymphocytic
spleen
which
accompanying
prognostic
an
sign
previously
cells
were
angles
to the surface
sometimes
branched,
The
cytologic
character-
similar
had
to
imply
a
the
to
reticulum
wet
Phase
in
that
the
the
ring
in
contrast
cell
similar
al.,3
the
the
in
with
study
cytologic
microscopy
demoncannot
stained
smears.
The
slip
may
of
and
which
cover
its
reticular
characteristics
blood
of
cell”
of lymphocytes
flagellated
aid
she
bodies
reticular
observed
smears
cytologic
difficulty
limitation
its
the
reticuloendotheliosis.
by Bouroncle
et
cells.
cells
over
“phagocytic
Sundberg
of
“phagocytic
Furthermore,
cells
and
correspond
means
described
lymphocytes.
that
chambers.
with
apparent
a firm
sufficiently
reticular
from
leukemic
et al.1 and
reticulum
asvmptomatic
an
chronic
at right
a thin,
had
of cytoplasm
may
by
ring
is possible
extensions
films
Mitus
therapy,
flagellated
cells
Both
types
of
Sundberg12
of
in slide
blood
out by
iii
Finkbeiner.’#{176}
blood.
were
nodes,
It
these
of cells.
by
nodes
diagnosis
in the
or
2 types
minimal
was
radioresistant.
cells
extensions
lymph
cells
in stained
As pointed
strates
the
lymphocytes.
Sundberg
were
surrounded
imprints
smear
in the
appearance
lymph
flagellated
of human
observed
living
cells
and
reticular
seen
cells
Despite
splenomegaly
be a favorable
of
observed
and
remained
al.,
is the
view
Durant
symptom
and
et
form”
were
with
who
cells”
Gosselin
reported
stage
crest.
Massive
disease
may
hairy
to
and
also
a terminal
similar
in
hyperplastic
Both
hairy
relationship
the
for
were
of
by
Reich”
predominating
chronic
patients
“pure
splenic
benign.
In
of symptoms
in
and
were
was
in their
lymphoid
remissions
with
numerous
cytoplasmic
projections
cells.
Some
of the cells of both
types
crescentic
In
be
the
that
the
the most
splenomegaly.
years.
disease
with
leukemia”
remissions
cured
the
disease
“spontaneous”
massive
lymphocytosis,
The
lesions
According
belief
and
lymphocytic
experienced
apparently
the
disease
that
patients
of
of
no palpable
15 years.
splenomegaly.
expressed
of this
interest
course
most
in
spleen
with
relative
in the
blood.
10 to
reticuloendo-
features
of the
chronicity
of the
reticuloendotheliosis
massive
“chronic
their
patients
and
of
is
with
of
the
had
have
form
it
clinical
enlargement
survived
the
“leukemic
significant
The
of whom
were
Italian
most
diagnosed
to severe
radiosensitive.
some
of
The
moderate
initially
mCst
patients
no severe
anemia,
leukopenia
and
reticular
lymphocytes
in 22 patients,
of
ill
of tile spleen.
of the patients
DONNELLY
diagnosis.
Gosselin
in
chronically
to radiotherapy
results,
but
AND
squash
be
wet
and
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
HALRY”
CELLS
distort
of
the
time.
and
leukocytes
and
Furthermore,
in
examine
The
methods
the
counts
cells
the
slide
that
the
for
usually
remained
addition,
the
hairy
cells
not
chamber
Additional
was
that
cytologic
particular,
cell
survived
to
because
were
During
allowed
from
examinations
2
of
projecting
cells
had
one
or more
on glass,
with
did
not
r.
2000
lymphoid
Both
patient
is
now,
numbers
The
pathologic
in
cells
were
Mitus
relatively
cells
had
small
nucleoli.
The
were
identical
to the
of
del
in
round,
flagellated
examine
inusual
In
hairy
cells
resistant
leukemic
were
The
were
he
and
seen
in
the
short,
some
oval
cells
of
thin
these
or crescentic,
did
resistant
identical
nonblood
not
flatten
to irradiation
to
the
neoplastic
large
spleens,
moderate
anemia,
the spleen
of one patient
resulted
decrease
in the size
of the spleen.
21
months
by
after
diagnosis.
splenectomy.
Hairy
cells
The
He
are
still
is
and
in a
This
second
asympto-
present
in
small
patients.
in
the
blood
were
hyperplastic
number,
oval
cells
“phagocytic
SUMMARIO
In le curso
non-leucemic,
slide
were
patient,
nuclei
and
relieved
several
few
and
the
numerous
one
nucleoli.
to
diagnosis.
both
from
projections
the
et al.’
cells
after
of
hairy
suspensions
that
cell examination.
in test tubes.
were
by
In
The
particles,
was
blood
In
seen
of
and
many
cells
cells.
considered
asymptomatic
11 months
in the
seen
plasmic
of
thrombocytopenia
matic
it was
that
from
small
latex
hairy
and
cells
flatten.
disadvantage
macrophages,
“hairy”
masses.
Radiotherapy
and
a delayed
alive
good
hairy
to
and
mm.)
in
CONCLUSIONS
the
had
were
cells
with
the
a
cells.
(1
and
tendency
characterized
of
some
cells
patients
patient’s
were
phagocytize
thrombocytopenia.
prompt
leukopenia
of
deep
microscope
for
cell cultures
leukocytes
cytoplasmic
reticulum
and
of
viable
that
The
intp
called
surface
and
The
numbers
determination
AND
cells
the
placed,
preparation
visualized,
by
transform
cells
These
from
eccentrically
well
the
viable
peculiar
patients.
villi
The
sufficiently
no
(3)
x-irradiation,
roentgens.
2000
patients,
or
a leukocyte
large
remained
particles.
SUMMARY
leukemic
it was
it required
an inverted
data
were
obtained
not
of
little
latex
did
find
chambers,
as
demonstrated
had
villi
such
and
chamber
phagocytize
with
period
to
of
slide
incubation.
distorted
and
cultures
short
preparation
special
examination
used
not
short
2 to 3 weeks,
irradiation
after
slide
numerous
in
reagents
the
was
The
a relatively
is time-consuming
(1 )
cells
without
cells
spherical
did
the
facilitated
days.
it
involved
of
were
only
of leukocytes.
and
chamber
survive
leukopenia,
study
viable
leukocytes
condition
this
with
suspension
A special
so
in
cells
of
examination
of
ietmkocyte
the
cases
number
used
(2 )
of
(4)
that
a sufficient
suspension,
culture
209
IN BLOOD
large,
similar
lymph
with
to
The
numerous
nuclei,
long,
or
crescentic
were
resistant
to irradiation
reticular
cells”
of Sundberg.12
IN
“flagellated”
nodes.
some
cells
flagellated
thin
cyto-
of which
had
and
probably
INTEBLINCUA
de viabile
leucocytos
celiulas
designate
ab numerose
como
“capillute”
patientes
esseva
leucemic
notate
in
From www.bloodjournal.org by guest on June 18, 2017. For personal use only.
210
SCHREK
he sanguine
ab
curte
e tenue
certes
del
nucleos
illos
vitro,
patientes.
Iste
projicente
ab
mentionate
esseva
habeva
illos
contra
con
duo
villos
cellulas
oval
non
amiemia
tin del
de grado
patientes
Ic magnitude
partir
esseva
moderate,
resultava
del
11 menses
splen.
a partir
numeros
Q ue
nucleos
“flagellate”
se
con
provava
que
vive
del
e es
Cellulas
in
splenes
le “phagocytic
cellulas
allargate,
del
splen
declino
de
in
21
menses
a
tardive
“capillute”
le sanguine
plure
esseva
hyperplastic
relativemente
pauco
e tenue
projectiones
del
identic
esseva
et al.
ambes
persiste
in
patientes.
ab
resistente
inter
patiente,
le thrombocytopenia
patiente
vive e es asymptomatic
diagnose.
“capillute”
certes
Le
e certes
le cellulas
de Mitus
habeva
le secunde
Etiam
iste
de ambe
patientes,
cytoplasmatic.
eccentric,
asymptomatic
In
numerose
del
non se applattava
al superficie
de
latex,
e illos se provava
resistente
concludite
patiente
tempore
esseva
longe
numerose
crescentiforme
de
massas
location
Iste
in suspensiones
flagellate
o plure
DONNELLY
per
In un
Radiotherapia
e in tin
cellulas
vidite
cellulas
e haheva
del
cellulas.
e thrombocytopenia.
in un prompte
leucopenia
in le sanguine
pathologic
“flagellate”
un
lymphoidoreticular
cellulas
“capillute”
del tempore
del diagnose.
alleviate
per splenectomia.
micre
identic
r. Esseva
characterisate
del
e de
Le cellulas
particulas
a 2000
le neoplastic
cellulas
Le duo patientes
con
esseva
habeva
o crescentiforme
micre
nucleolos.
phagocytisava
irradiation
cellulas
le superficie
AND
numerose;
cytoplasmatic
quales
habeva
contra
irradiation
reticular”
simile
nodos
micre
illos
esseva
e ronde,
nucleolos.
e esseva
al
cellulas
lymphatic.
Le
Le
grande
oval,
o
cellulas
prohabilemente
de Sundberg.
ACKNOWLEDGMENTS
We are grateful
to Dr. Paul
Heller,
Chief,
Hematology
Section,
Veterans
Administration West
Side Hospital,
Chicago
for allowing
us to study
patient
Y, and for his kind interest,
cooperation,
and suggestions.
Dr. Noel Browdy,
Resident
in Medicine,
Veterans
Administration
West
Side
Hospital,
aided
in the preparation
of the clinical
report
of patient
Y.
ADDENDUM
Both
are still alive
patients
and
asyniptomnatic
28 and
18
months
after
diagnosis.
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1966 27: 199-211
"Hairy" Cells in Blood in Lymphoreticular Neoplastic Disease and
"Flagellated" Cells of Normal Lymph Nodes
ROBERT SCHREK and WILLIAM J. DONNELLY
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