Splenectomy in sarcoidosis

Splenectomy ARNOLD

From tbe Jackson Clinic,

hfudison,

in Sarcoidosis

S. JACKSOK,

M.D.,

Wisconsin.

N 1948 the National Research Council defìned disease sarcoidosis as a disease of unknown etiofogy with pathologie changes similar to those seen in tuberculosis. The condition may involve almost anJ- part of the body but is more commonly observed in the Iungs or in the cervical Iymph glands. S-rays of the chest show typical changes in the Iung fierds, such as symmetricaffy enlarged hiIar Ivmph nodes, soft peripheral infiltration an d GiIiary foei: often confluent or nodular Iesions or fìhrotlc scars in late stages. The intracutaneous tuberculin test is invariabl) negative. EpitheIioid cel1 tuhercles with caIcified bodies in giant ceffs are seen on examination of biopsy specimens of Iymph gland and skin. The Nickerson-Kveim test of an intracutancous injection of a preparation of human tissue obtained from a sarcoitf fesion results in a rcaction similar to that of the tuberculin test aftel>Iantous.. The disease runs ÍI chronic and unprecfictablc course. Spontaneous remissions occur, and in trvo of the cases involving the cervicnl glands that 1 have observed the patients now have remainetf \velI for ten ycars or more. The prognosis in the pulmonary type is Iess favorable, anti cleath may result liom frhrosis ancl subsequcnt respirator), nnd carcfiac invofvement. Until the advent of cortisone antf ACTH, treatment \vns Iargefy s,vmptomatic. Ho\~ever, these cfrugs have now been proved to be ol Iimitecf assistance. Rippmann [IJ] stated that the chief vafuc of cortisone ancf ACTH is (1) to carry n patient through an active stage to a spontaneous remission, (2) to speecf up the process of scarring of the sarcoid Iesions, nncf (3)to give the patient a suhjective feeling of well-being and to reIie\-e him from harassing symptoms. This author also

1 the

Anwil
802

\\ iSCYJl~.Till

i\~ladison,

stressed thc importancc of psychotheraplbccause of the chronicity of the cfisease, in g>ving the patient assurance through thc long period of ilfness. The spleen, along n-ith the liver, is said to be second on!‘; tc) IJ-mph nodes and lungs in frcquenc!. ot rn\,olvemcnt 1,~ this tfisease. Swera1 instances n-ith ancl wrthout splenomegal> onIJ- after splenectom>and were discovcrcd histologie examination of the spleen in lxtients in \vhorn the cliseasc WRS unsuspcctetf preoperati\ el). It is gcnerall\- agreetl f>y those \vho ha\pc cxtl cortisone and ACTH in snrcoidosis that these drugs as

hasten

shown

the

hcnling

in size and nurml,er Ray

\ioj

hccausc

to

thrcc

1 n two

~vas present;

v c’ll to

in

of the tuberclcs.

of splcnotmegaI~-.

spondccl

fesions

inci-case

and f,y the cleïreas(

g:l\-e cortisone

h>persplenism othcr

13~ the

2nd fIJ alinization

fihrosis

third

of the sarcoid

histofogically

steroicl

paticnls

of these

one

c’:lses

patient

1 hcrap\,

hut

in

rethc

c:iw splcnectoni~ ~vas neccssar~~. Tlic patient rcïcived thc drug fvxiusc of ;i

gre:itI,v

enf:lrgecf

decrc:aetl

splwn.

in six

In this

case the spleen

approxiimatel>-

onv-thircl

c)n

ther:II,> 1lillcr noted

ancl Bass

21 Ii\~oraf)le

sone ancl ACTfl pulnionark

[ql ancl Shulnian response

to thc

in sarcoiclosis,

and cy-c fesions

of the per’iphera1

I>,niph

hut

ct af. [/;j use of cort i-

not cinf> i11 the ais0

in tfitl

sizc

nodes and spleen.

In scveti of thc ninc cases in which tlic spleen \vas enlargccl,

8 clecreasc in size

li~llov ing tlw

use of ACTH was notecl I>J Shultmnn ct al. Thtx dccrcasc in sim \vas olxer~wl as t:rrli 2s tfie sccond tla~- t‘ollon-inp ther:ip~~. 1 n scvcn cases thc spleen coul
Splenectomy

in Sarcoidosis

In these cases the pathoIogic fìndings were aImost entirel?. confined to the spleen, and in one of Kay’s cases the spleen weighed 4,800 gm. Lovelock and Stone [6,7] concluded that a majority of patients with pulmonary sarcoidosis show some roentgenographic evidente of improvement following therapy with cortisone or ACTH, hut no criteria have been found which permit US to predict the extent of such improvement.. A higher percentage of clearing was noted in patients receiving cortisone or ACTH (64 per cent:1 than in the untreated cases (44 per cent). RoentgenoFraphic evidente of clearing is not aIwa‘-s mamtained, however, as indicated 65 one patient who had rapid recurrence after each of two courses of cortisone therapy. Siltzbach [IG] concluded that cortisone and corticotrophin generaIIy act favorabIy upon intrathoracic sarcoidosis, aIthough the benefits usuall,v prove temporary. In chronic pulmonary sarcoidosis the effects of the hormones are variable. Sometimes symptomatic relief is dramatic anci sustained. More often the reIief is partial, and relapse occurs promptly after the hormones are withdrawn. However, in two cases of splenomegaIy treated by this method, no reduction in size was seen although the drug was used for sixty-three days in dosage of IOO to 150 mg. dailh-. The cases did seem to improve generally. In 1954 Kay and Royster [dc] reported on the use of cortisone in the treatment of sarcoidosis of -the spleen. In this condition the spleen can attain great size, causing pressure on al1 the adjacent organs and consequently requiring spIenectom>-. These authors found that the risk of splenectomy couId be reduced by the preoperatk-e use of cortisone to reduce the size of the spleen. CASE

FIG. I. LongitudinaI cross section of spleen iIlustrating tbe grerrtly increased thickness of the organ. Nute the mottled appear~~nre produccd by the conffuetw oj‘ the snrcoid nodulcs.

cian’s care and received a course of cortisone, 25 mg. every eight hours, with no appreciable change in the size of the spleen after ti\-e weeks. The dosage of cortisone was then doubled, and two months later it was noted that the spleen had been reduced to 3 fingerbreadths belon- the umbilicus and to the midIine, a considerable reduction from the time of the fìrst examination. The cortisone was stopped and -thcre appeared to bc no indication for splenectomy at that time. However, on April 29, 1954, seven months after admission, splenectomy was advised but was refused. At this time moderate enlargement of the liver was also noticed. Tn-o months later the patient reported feeling “run clo\yn” hut again refused surgery. NO change in the size of the spIeen was noted. A secondary anemia was now present with hemoglobin, IO gm. per cent, and the red bIood cel1 count 3,100,000 per CU. mm. Bloed transfusion n-as advised, to be foIIowed by splenectomy. The operation was performed on September 20th. Through a Ieft rectus incision the spleen was espIored and found to be approximately ten times norrnal size. (Fig. I .) Crossl‘, it. had a mottled appearance, undoubtedly thc result of sarcoidosis. There were numerous adhesions to the diaphragm, colon, stomach and pancreas. Operation was dificult because it was not possibIe to isolate the splenic artery after opening the gastrocolic omentum. The \-essel was matted in many gIands and adhesions. The tail of the pancreas was ligated near its attachment to the spleen. Operation was accomplished

REPORT

The patient, a thirty-nine year old woman, was referred to the Clinic on September 23, 1953, because of an enlarged spleen. With the exception of the splenomegaly, no abnormality \vas noted hy the examining physician. Bone marrow examination and splenic puncture were negative. AI1 other Iaboratory examinations were negativc. S-rays of the lungs, however, were characteristic of earIy sarcoidosis, and this was considered the etiology of the splenomegaly. Inasmuch as the patient was experiencing no particular discomfort from the enIarged spleen at this time, she was sent home to her physi803

Jackson u.ith approximately no bloed Joss clespite thr: handicaps. The liver presented the same gross appearance as the spleen hut did not appear appreciably enlarged. The stomach, colon ancl other viscera appeared normal. The postoperativc course was uneventful and the patient \vas discharged on the twelfth tlay. The pathologist, Dr. Etheldred Schafer, reportecl : “The specimen consists of a spleen weighing 2,400 pi. ancl measuring 29 J>y 20 and up to 8 cm. in maximum thickness. The capsule is tense, slightly thickened, Jout a translucent, pale gra?’ except lvhere mottled \\,ith vellonish gral-, opaque foei and roughened b> hbrous tags; some of these are delicate anti easily broken. Over the upper pole there is a slight granularit? to the capsule, individual granules measuring from r to 2 or 3 mm. in maximum diameter. The previousJ>~ mentioned ~;ellowish-grah- foei are slightly tlepressed. Sectrons are made with exceeclingly great diffrcult>through the Jong axis to reveal slightly hulging, light red, cut surfaces having a meaty appearante and mottlecl \i-itfi stellate gra>- foei ancl dark recldish-purple areas. Thc fat at the hilus of the spleen contains a numher of enlargecl, soft, pinkish-red Jyrnph nodes and an irregularly shaped piece of pale pinkish-tan, fÌnel>Johulated, pancreatic tissue measuring 3 by 2. j 1>\- 2 CIll.

description: iFluJtiple sections ’ “1Iicroscopic show complete ohliteration of the splenic architecture by a granulomatous type of inAammation characterized hy wel1 circumscribed, circular or oval Jesions composed of epithelioid cells, multinucleated giant cells, and smal1 Jymphocytes. Most of these Jesions are free of centra1 necrosis, hut there is extensive necrosis in the intervening portions of the by hemorrhage, mixed tissues, accompanied infJammatory cells and hyalinized fibrosis. The cytoplasm of some of the giant cells is vacuoIated; this Ied to an extensive search for a Histoplasma capsuIatum, hut none were noted. The necrosis is thought to be secondary to circulatory disturbances by the extensive involvement of the spleen hy the above Iesions and not an integral part. of the granulomas. “Sections of the accompanying Jymph nodes show similar tubercle-like lesions without centra1 necrosis. There are numerous irreguIarly shaped, whorIecJ, acidophilic masses between the tubercles. In the intervening tissues, there are numerous Jymphocytes, plasmacytes,

ancl :I l& rosinopfiils. Ilacroph;ycs :Irc* qui tc nunierous; somv of these contain golden tx-01~ n pigment, ethers ha\.c fo:rrn) CJ toplxm. Asteroitl

Mies

suggcstion

;u-c

“Diagnosis: fIamnint,ion

nat

01’ oitv

rJote
nltfJou~lJ

in ;\ giaiit

c,ell.

konspecific

t.lwrc.

granL~lonJ~~tous

is ;I

in-

spleen and hiJar 1~mph nodcs clinical ~Jiwgnosis Ijf \r.it,h thc of

consistent sarcoidosis.” On sc\pernl subsequent l,isits to thc Clinic thc paticnt bas heen observccl vy Dr. JI. (:. Ashman, ;ind her gcneral conclitron lias remained satisl’actor,y. TJw I~Jootl munt JMS rcmaineti 110 ahmn~l~kieS Of kiJl,X dm’ normal, :lJld orgaris liaw been notecl, altliough tlic lil cl continues to be sJightl>. cnJarged.

There is no particular reason for performing splenectom>. in sarcoidosis escept 11here tllc spIeen reaches siicli size as to encroach upon and embarrass thc other organs. Because this cvndition bas rarely lwen notcd in the

litcrature,

this

cuse

is reportecl.

The prcoperati1.e

beneficial effect ofcortisont in recluc-ing the size of the spleen, as previousl>reported 1,) IGr>- anti Roystcr, is reconfìrmed.

6. Lov~~oc~. F. J. and S.I.ONE, D. J. Cortisonc. thrrapy of Boeck’s sarcoid. J. A. M. A., 147: 930. 1951. 7. Lowroc~. F. J. :rnd 50s~. D. .l. The therapv of

Splenectomy

in Sarcoidosis in reIation to ACTH therapy. BuU. Joltns H(qkins Hosp., 91: 345, 1952. Buil. Geisinger 12. RIPPMANX, E. T. Sarcoidosis. 1Víemorial Hosp. PYFoss Clin., 8: 142, 1956. 13. SHULMAN, L. E., SCHOENRICH, E. H. and HAKVEY, A. M. The effects of adrenocorticotropic hormone IACTH) and cortisone on sarcoidosis. BuU. Jobns Hopkins Hosp., 91: 371, 1952. 14. SILTZBACH, L. E. Effects of cortisone in sarcoidosis; a study of thirteen patients. Am. J. Med., 12: 139, 1952.

In pulmonary form of Boeck’s sarcoid and its modification by cortisone therapy. Am. Reu. Tuberc., 67: 164, 1953. 9. MILLER, M. A. and BASS, H. E. Effect of acthar-C (ACTI-I) in sarcoidosis. Ann. Int. Med., 37: 776, 1952. 10. RAV, E. S. Sarcoidosis; its diagnosis and management. W’est Virginia M. J., _52: 200, 1956. II. RILEY, R. L., RILEY, M. C. and HILL, H. McD. Diffuse pulmonary sarcoidosis; diffusing capacity dwing txcrcise and other lung function studies

805