Annals of the Royal College of Surgeons of England (1988) vol.
70
Elective splenectomy
disorders
I R GRANT MRCP(UK) MRCPath
Senior Registrar in Haematology
S W PARSONS FRCS FRCSEd
Registrar in Surgegy
J M S JOHNSTONE FRCSEd
Consultant Surgeon
J K WOOD FRCP FRCPath
Consultant Haematologist
Departments of Haematology and Surgery, Leicester Royal Infirmay
Key words: SPLEINEICTOMY; HAEMATOLOGICAL DISEASE
Summary
We reporl on 106 elective splenectomies performed for haematolo-
gical disorders belween March 1979 andJanuary 1986. The most
common indications were immune thrombocytopenic purpura (30
patients) and Hodgkin's disease (19 patients). However, staging
laparotomy is no longer performed routinely for patients with
Hodgkin's disease and the reasons for this are discussed. Other
indications for splenectomy included splenic pain (13 patients),
autoimmune haemolytic anaemia (12 patients), hereditary sphero-
cylosis (1I patients) and hypersplenism (9 patients). The overall
morbidity and mortality was 48% and 5% respectively. The most
common postoperative complication was thrombocytosis (defined as
a platelet count >800X 109/1) and occurred in 26 patients. This
review confirms that splenectomy continues to have an important
role in the management of certain haematological disorders.
Introduction
Thc first clective splenectomy in Britain was performed
by Spencer Wells in 1866 (1). A series of 29 cases was
subsequently reported by Collicr in 1882 (2). Since then
splenectomy has had an important though changing role
to play in the management of haematological disease.
Later it played a part in the staging of Hodgkin's disease
and to a lesser extent in non-Hodgkin's lymphoma and
has made a significant contribution to our understanding
of the pathology of Hodgkin's disease.
In this review of 106 consecutive splenectomies, the
important and changing role in relation to Hodgkin's
discase of this operation is illustrated. Operative tech-
nique, morbidity and mortality are discussed. Attention
has been given to the purpose of the operation and
whether or not the operative aim was achieved.
Correspondence to: J M S Johnstone, Department of Surgery,
Leicester Royal Infirmary, Infirmary Square, Leicester LEI
5WW.
in haematological
Of particular concern and interest are those patients
with lymphoproliferative and mycloproliferative dis-
orders. In such patients splenectomy is a major proce-
dure and it is important to confirm that this group of
patients both benefit from operation and obtain reason-
able life expectancy in which to enjoy such benefit.
Methods
The notes of 106 patients who had undergone
splenectomy at the Royal Infirmary for haematological
disorders between March 1979 and January 1986 were
analysed retrospectively to determine age, sex, diagnosis,
morbidity and mortality and to establish in particular
whether the intended benefit from surgery was achieved.
This number represented all cases where splenectomy
was conducted for haematological disorders where the
catchmcnt population was 850 000. Eighty three were
performed by one surgcon (JMSJ). Patients were refer-
red predominantly by hacmatologists with a small num-
ber (9) from radiotherapists, all of these for staging
laparotomy for Hodgkin's disease. Twenty one
splenectomies were performed in March-December
1979, 16 in 1980, 19 in 1981, 16 in 1982, 15 in 1983, 14 in
1984, 2 in 1985 and 3 in January 1986. The respective
figures for Hodgkin's disease were 6 in 1979, 6 in 1980, 4
in 1981, 0 in 1982, 2 in 1983 and 1 in 1984.
The indications for the splenectomy are given in Table
I. Splenic pain is the only indication where a symptom
rather than a disease process is used to justify
splenectomy. Of those operated on for pain, all 5 with
lymphoproliferative disorders had non-Hodgkin's lym-
phoma and of 8 with myeloproliferative disorders, 7 had
myelofibrosis and one had chronic mycloid lcukaemia.
All these patients had massive splenomegaly.
Preoperatively particular attention was paid to the
need for re-introduction of steroids and whether blood
30 I R Grant, S W Parsons et al.
TABIL;I Summary of the indications for which spleneclomy was performed with oulcome
No. with Early (<30 days)
enlarged spleen postoperative Aim
Indication for No. at operation deaths Late deaths achieved
splenectomy (n= 106) (n=55) (n=5) (n=24) (n= 79)
Immune thrombocytopenic 30 1 I at 10 days- 21
purpura myocardial infarct
Hodgkin's disease 19 6 1 at 5 months - progressive disease 19
(staging) 1 at 8 months - progressive disease
1 at 3 years - chest infection and
disease
1 at 1 year - overwhelming sepsis
1 at 1 year - progressive disease
Splenic pain (n= 13)
Lymphoproliferative 5 5 1 at 11 months - disease 5
1 at 20 months - pulmonary embolism
following below
knee amputation
1 at 23 months - disease
Myeloproliferative 8 8 1 at 18 days- 1 at 15 months - disease 7
major gastro- 1 at 24 months - pneumonia
intestinal bleeding 1 at 26 months - gastrointestinal
bleeding
1 at 27 months - cerebrovascular
accident
Autoimmune haemolytic 12 5 1 at 14 days - 1 at 5 weeks - chest infection 3
anaemia major gastro- 1 at 2 years - abroad ? haemolysis
intestinal bleeding 1 at 3 years - haemolysis
1 at 6 years - haemolysis

Hereditary spherocytosis 11 11 11
Hypersplenism (n=9)
Idiopathic hypersplenism 3 3 1 at 15 months - chest infection 3
1 at 8 months - cerebral vascular
accident
Lymphoproliferative 5 5 1 on first post- 1 at 3 years - progressive disease 4
operative day - 1 at 1 year - progressive disease
major operative 1 at 2 years - progressive disease
bleeding 1 at 3 years - progressive disease
Myeloproliferative 1 1 at 5 years - chronic renal failure
Hairy cell leukaemia 6 6 1 at 10 months - disease 3
Hodgkin's disease 1
(for removal of
bulk disease)
Felty's syndrome 1
Hereditary elliptocytosis 1 1 at 7 days- 0
presumed sepsis
Diagnostic 0
I
Autoimmune neutropenia 0 0
Systemic lupus 0 0
erythematosis

product support was likely to be necessary. Standard
laboratory investigations performed preoperatively in-
cluded a full blood count and platelets, urea and clectro-
lytes, liver function tests, electrocardiograph and a chest
X-ray. Prophylactic antibiotics were used immediately
preoperatively and for 3-5 days postoperatively but con-
tinued until the patient could tolerate oral pcnicillin V.
More recently subcutaneous heparin was given prophy-
lactically unless contraindicated by the underlying dis-
ease. Pneumococcal vaccine was not used.
Most operations were performcd by one surgeon
(JMSJ) through a left paramcdian incision. In a few
patients the splenic artery was ligated in continuity
2-3cm proximal to the hilum before splenic mobilisa-
tion. In most, however, the splecn was first mobilised by
division of the licnorcnal ligamcnt and the splenic artcry
 Elective splenectomy in haematological disorders 31
then ligated in continuity from behind again 2-3 cm disorder. Of particular interest, no patient developed a
proximal to the hilum. Once a safe situation had becn subphrcnic abscess.
achieved, splenectomy was performed in routinc manncr Fivc patients died within 30 days of operation. One
with ligation and division of the short gastric vessels and paticnt had a massive bleed in the immediate postopera-
dissection, ligation and division of the branchcs of the tivc period and died during rc-laparotomy following a
splenic artcry and vein in the hilum. A single 6.35 mm cardiac arrest. Hc was aged 56 and had non-Hodgkin's
Portavac® drain was left in the splenic bed. Dctailcd lymphoma with a grossly enlarged spleen. Splenectomy
search was then made for splenunculi and finally mcticu- had becn undertaken to reducc his transfusion require-
lous attention given to hacmostasis. A nasogastric tubc mcnts. The second patient died on the seventh postop-
was used routincly to prevent gastric ilcus. Standard crativc day from prcsumcd scpsis. Hc was aged 76 and
postoperative care was given with special attention paid underwent splenectomy due to dramatically increasing
to the rcspiratory system. A full blood count was carried haemolysis secondary to hereditary elliptocytosis. The
out daily and if the platelet count rosc abovc 800x10X/l, third dcath occurred in a 70 year old man with a
aspirin 300mg twice wcekly and dipyridamole 50mg mycloprolifcrativc disorder who had a splenectomy per-
threc times daily were started. Following dischargc an formed for splenic pain. He had a chcst infection develop
carly follow-up appointment was made to the referring on the third postoperativc day and a pulmonary embol-
unit's outpaticnt clinic. The paticnts werc seen at follow- ism on the eighth day. On the eighteenth day he had a
up usually oncc in a surgical outpatient clinic. large gastrointestinal blecd and had a cardiac arrest. The
fourth dcath occurred in a 63 year old man with fulmi-
nant autoimmune haemolytic anacmia. Hc had a large
Results gastrointestinal bleed 2 wecks postoperatively and died
Of the 106 paticnts 58 werc female and 48 were malc. following a rc-laparotomy wherc 2 prepyloric ulcers and
The mcan agc was 44 years (rangc 3-76 years) and the a duodenal ulcer werc found. The fifth death was in a 54
mcan hospital stay was 7.4 days (rangc 6-29 days). year old man who had a myocardial infarct 10 days after
Indications for which surgery was undertaken arc shown splenectomy performcd for refractory idiopathic throm-
in Tablc I and additional procedurcs shown in Tablc II. bocytopcnia at a timc when his platelet count was
All paticnts undergoing a staging laparotomy had liver 974X 10'3/1. In ncither paticnt dying of gastrointestinal
and lymph nodc biopsics. Fivc of 10 cholecystectomics blecding was a H2 antagonist used.
were performed in paticnts with hereditary spherocyto- Excluding carly postoperative dcaths, 24 patients have
sis. Splenunculi werc found in 17 paticnts with no pre- subsequently died; 20 with progressivc lymphoprolif-
dilection for onc disease. crative or mycloproliferative disease and 4 with autoim-
The causcs of carly morbidity arc shown in Tablc III. munc hacmolytic anacmia. The mean length offollow-up
They occurred in 51 patients and 16 patients had more of survivors is 44 months (rangc 1-84 months).
than onc complication. Fiftcn complications occurred in The number of paticnts undergoing splenectomy in
12 of the 35 paticnts who underwent splenectomy alone. which the outcome has becn accomplished as ofJanuary
As cxpected thrombocytosis, defined as a platelet count 1986 is shown in Tablc I. The aim was achieved in all
>800X 109/1, was the most common complication patients with hereditary spherocytosis and in thosc
followed by chcst infcction. Threc patients developed undergoing splenectomy as part of a staging procedure
pulmonary embolism; of these 2 had idiopathic throm- for Hodgkin's discase.
bocytopcnic purpura and one had a myeloproliferative Of 25 evaluable paticnts with idiopathic thrombo-
cytopcnic purpura (4 werc lost to follow-up and one was
TABIL, 1i Additional procedures (n=53) a postoperative dcath) 21 remain in remission at 1 to 82
months (mcan 40 months).
Cholecystectomy 10 Three of 12 patients with autoimmune haemolytic
Distal pancreatectomy (for large splenunculus) I
anacmia as of January 1986 are still in remission at 12
Exploration of common bile duct 2
Liver biopsy 19 months, 20 months and 30 months. Five others had a
Lymph node biopsy 19 temporary remission (range 5-24 months); of these 3
Pancreatic biopsy I have since died of fulminant haemolysis. Three patients
Sterilisation 1 never entered remission; 1 was an early postoperative
death, another with chronic lymphatic leukaemia died 6
TABLEiI i Causes of early (<30 days) morbidity in 51 patients wecks after operation of a chest infection and the third is
(n=69) alivc 22 months after operation but on azathioprine and
steroids. One paticnt was lost to follow-up at 8 months
Thrombocytosis (>800X101/1) 26 but was in remission at that time.
Chest infection 20 Three of the 6 patients with hairy cell leukaemia
Wound infection 4 remain in remission (range 2-26 months). Of the other 3
Haemorrhage 4 one relapsed at 12 months and 2 never entered remission;
Urinary tract infection 4 1 of these having since died.
Pulmonary embolism 3
Ischaemic colitis 2
Deep vein thrombosis I Discussion
Gastrointestinal haemorrhage I A recent review shows that nearly 50% of clectivc
Ileus 1 splenectomics werc performed as part of the staging for
Myocardial infarction I
Hodgkin's disease (3). This is not our experiencc as only
Pelvic abscess I
Wound dehiscence I 19 of the 106 werc done for this reason. Indecd, sincc
1982 only 3 staging laparotomies have becn done demon-
32 I R Grant, S W Parsons et al.
strating a decline in our centrc in enthusiasm for this
investigation. Our changc in policy rcflccts the changc in
attitudes ovcr the use of routinc laparotomics in Hodg-
kin's discasc for a number of rcasons (4,5). These includc
the wider usc of combination chemotherapy for limited
Hodgkin's discase and the introduction of computcrised
tomography and lymphangiography. Howevcr, somc
centrcs do still recommcnd the use of staging laparotomy
for ccrtain groups of paticnts with Hodgkin's discase (6).
The commonest postopcrativc complication was
thrombocytosis, dcfined as a platclet count more than
800X 109/1 (7). Although the risk of developing thrombo-
cmbolic discasc in this situation is not known, we prc-
scribc aspirin and dipyridamole as recommended by
sevcral groups (7,8) until the plateclet count rcturns to
normal. In this scries onc patient with mycloproliferative
discasc developed a deep venous thrombosis 5 days after
operation; her platclet count at that timc was normal.
Two paticnts with idiopathic thrombocytopcnic purpura
and onc with a mycloproliferativc disorder developed
pulmonary cmboli in the postoperativc period and again
cach paticnt had a normal platclet count. Two paticnts
had myocardial infarcts following splenectomy for
idiopathic thrombocytopcnic purpura. Onc died and his
platclet count was 974X 10'/l at the timc of the infarct. It
is possiblc to speculate that the infarct was precipitated
by the thrombocytosis and this may havc contributed to
his dcath.
Chcst infcction was the second commoncst postopera-
tivc complication. However, the diagnosis madc on the
basis of chest X-ray and fevcr is difficult to judge in
retrospect. Therc werc only 4 positivc sputum cultures, 2
Haemophilis influenzae and 2 Staphylococcus aureus. Basal
atclectasis is common aftcr major abdominal surgery and
the fevcr may havc becn part of thc patient's underlying
discasc.
The splecn plays an important rolc in immunity and
its removal impairs certain host defence mechanisms.
Thesc include the lowering of IgM levels (9) and a defect
in the complement pathway rcsulting in impaired scrum
opsonic activity which is important for efficicnt phagocy-
tosis of encapsulated organisms such as the pneumococ-
cus (10). For thcsc reasons ovcrwhelming scpsis from
cncapsulated bacteria occurring months to years after
splenectomy is a well recognised complication (11). In
this rcspect it is intcresting that in this scrics the wound
infection rate was only 4% and there was no subphrenic
abscess, prevented wc fccl by meticulous attention to
surgical technique. Furthermorc, there has so far becn
no confirmed casc of ovcrwhelming postsplenectomy
infcction, although scpsis was a possiblc causc of early
death in one paticnt and a contributary cause of latc
deaths in a further 4 patients. Bccausc of the long-term
risk of infection wc usc lifelong pcnicillin which has
previously been shown to bc cffcctivc although patient
compliance may bc a problem (12). In this scrics
pneumococcal vaccinc was not routincly administcred.
However we now routincly give the 23 valent vaccinc
along with lifelong penicillin as the usc of both has
recently been emphasised (13). If possible the vaccinc
should be given several weeks prior to splenectomy sincc
removal of the splecn diminishcs antibody response (14).
Twenty-onc of our 25 paticnts (84%) with idiopathic
thrombocytopenic purpura arc off steroids with normal
platelet counts and this rcsponsc ratc is in close agrce-
ment with other centrcs (15,16). The splenectomy was
usually performcd if the paticnts failed to respond to an
initial course of steroids or if therc had becn one rclapse.
Splenic pain, duc to the physical sizc of the organ or
infarction, is an acccpted indication for operation and we
have operated on 13 patients for this reason (17). Both
lymphoprolifcrative and mycloprolifcrative disorders
may rcsult in a vcry largc splecn and lead to splenic
infarcts resulting in sevcrc pain. In paticnts undergoing
splenectomy for mycloprolifcrative disorders there is an
incrcased risk of morbidity and mortality (18), with the
operative mortality as high as 25% in paticnts with
myclofibrosis (19). Thcsc paticnts arc usually elderly,
often with markedly enlarged splecns making the opera-
tion more difficult, and the discasc is associated with an
incrcased risk of cither blecding or thrombosis duc to
qualitativc plateclet defccts.
Our results in paticnts with autoimmunc hacmolytic
anacmia show that it can be a far from benign diseasc.
The 3 that died of fulminant hacmolysis all had 'mixed'
type autoimmunc hacmolysis with both IgG and complc-
mcnt coating of the red cclls with a cold antibody
rcacting at 30°C or abovc in saline. Mixed type auto-
immunc hacmolysis has becn prcviously described as
following a sevcre clinical coursc (20). Only one
splenectomy was performcd for hacmolysis associated
with cold antibodics as it is rarely beneficial (21), this
paticnt remains in remission 4 years latcr. Similarly, no
splenectomics werc pcrformcd for drug induced
haemolysis. We do not routinely perform splenic sequcs-
tration studies sincc paticnts may often benefit from
splenectomy cven if therc is no significantly raised
splecn/livcr ratio demonstrated (22).
Hairy ccll lcukaemia prcsents with a pancytopcnia
and a large spleen. Splenectomy often results in recovery
of the peripheral blood counts, sometimes for prolonged
periods. For thosc that subsequently rclapsc intcrferon is
becoming an accepted method of treatmcnt (23). Wc
have used intcrferon in onc of our patients when shc
relapsed 12 months following splenectomy with subse-
quent recovcry of her peripheral counts.
Overall the operativc aim was achieved in all patients
undergoing splenectomy as part of the staging procedure
for Hodgkin's discasc and for hereditary spherocytosis.
Furthermorc 12 of 13 patients with splenic pain and 8 of
9 paticnts with hypersplcnism bcncfited from
splenectomy. Three-quarters of patients with autoim-
mune haemolytic anacmia had an initial response but
relapsc rates are often disappointingly high, up to 80%
(24), and our figurcs of only three of the twelve still in
remission rcflcct this. Howevcr, once relapse occurs the
steroids dosagc required to maintain acceptablc hacmo-
globin levels may be lower than beforc splenectomy.
Five paticnts died within 30 days of operation. Other
scrics report mortality rates of 0-5% (3,25,26). The
operation can bc difficult and calls for an experienced
surgeon. Two postoperativc dcaths werc from stress
ulccration and ill paticnts would now be given H2 anta-
gonists prophylactically.
Dcspite the cfficicncy of splenectomy as a therapeutic
tool in the types of hacmatological disorders reported
herc, it is still important to weigh the potential benefits
against possiblc morbidity and mortality when consider-
ing a patient for splenectomy. The indications and rela-
tive contraindications must be carefully judged in each

casc. In general in the elderly patient unnecessary delay
is to be avoided once a decision to proceed to splen-
ectomy has becn made. Full discussion of the procedure,
its likely bencfits and possible complications must be
held with the paticnt and his rclatives. In some condi-
tions (hereditary spherocytosis) cure is ccrtain, in others
(myclofibrosis) bencfit is not guaranteed.
The era of diagnostic laparotomy and splenectomy in
lymphoproliferative disorders may be coming to an end
with the advent of grcatcr sophistication in non-invasive
imaging tcchniques. An occasional complex case will still
justify such an approach, particularly when lymphoma is
strongly suspected and tissue may only be available from
an cnlarged spleen removed at operation.
Finally, wc would stress that although morbidity and
mortality are relatively high in patients undergoing
splenectomy for splenic pain, particularly those with
mycloproliferativc disorders, all our paticnts gained
symptomatic rclief. They also decrcased their transfusion
requiremcnts postsplenectomy, with reduced hospital
visits and the combination of thesc significantly im-
proved their quality of life.
We are indebted to Dr R M Hutchinson and Dr V E Mitchell
of the Department of Haematology; to Dr S Khanna and Dr FJ
F Madden of the Department of Radiotherapy and Oncology
and to Professor P R F Bell, Mr N Everson, Mr D P Fossard
and Mr D F L Watkin of the Department of Surgery for
allowing us to include their patients in this review.
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Received 2 June 1987