Splenomegaly

Schistosomiasis
This is prevalent in Africa (particularly around the Nile delta), Asia and South America.
It is caused by infection with Schistosoma mansoni in nearly 75 per cent of cases and by
Schistosoma haematobium in the remainder. Splenic enlargement is produced by
hyperplasia which is induced by phagocytosis of disintegrated worms, ova and toxins,
and by portal hypertension which is the result of hepatic fibrosis.
Clinical features
Splenomegaly arising from schistosomiasis can occur at any age and is more prevalent in
males. The degree of splenic enlargement reflects the extent of hepatic fibrosis and may
be massive.
Investigations
The urine and faeces are examined for ova. Liver function tests reveal a varying degree of
hepatic impairment. A hypochromic anaemia is always present.
Treatment
Successful medical treatment of established cases does not result in regression of the
splenomegaly. Removal of the painful and bulky spleen is indicated where there is no
evidence of hepatic or renal insufficiency. If ascites is present, a portosystemic shunt
should be combined with splenectomy.
Tropical splenomegaly
Massive enlargement of the spleen occurs frequently in the tropics, for example in
malaria (especially in children), kalaazar and schistosomiasis (see above). In parts of
Africa and New Guinea splenomegaly cannot be fully attributed to these diseases because
tropical splenomegaly is restricted to only a few adults in areas where malaria is endemic.
The most likely explanation is an abnormal immune response to malaria or unusual
species of plasmodia. Malnutrition may also be a factor and there is a high incidence in
lactating women.
The spleen is grossly enlarged (2000—4000 g). This is associated with anaemia due to
shortened red cell life and thrombocytopenia due to splenic sequestration of platelets,
which respond to splenectomy. Splenectomy is indicated for those disabled by anaemia
or by the weight of an enormous spleen. Splenectomy reduces immunity to malaria and
therefore antimalarial chemotherapy (e.g. proguanil) should follow splenectomy in
malaria endemic areas and be maintained for life.
Hypersplenism due to portal hypertension
Splenomegaly invariably accompanies portal hypertension. This splenic enlargement
results in thrombocytopenia (due to splenic sequestration of platelets) and
granulocytopenia. These are permanently relieved when splenectomy accompanies
operation for the relief of portal hypertension. Shunt surgery alone does not have the
same effect.
Felty’s syndrome
A moderate number of patients with chronic rheumatoid arthritis develops mild
leucopenia; in a few of these, neutropenia becomes extreme and is usually associated
with enlargement of the spleen; this combination is referred to as ‘Felty’s syndrome’. A
remarkable characteristic of this syndrome is that the leucopenia and splenic enlargement
are apparently unrelated to the severity of the arthritic changes; indeed, in some instances
the arthritis has begun to improve or has become quiescent by the time the low white cell
count and the splenomegaly become unmistakable. In those cases in which the arthritis is
slight but the splenic enlargement and blood changes are much in evidence, a diagnosis
of primary splenic neutropenia is sometimes made. The results of splenectomy are
variable. Usually there is an improvement in the blood picture with increased neutrophils,
but this improvement is not maintained. However, the liability to infections seems to be
decreased in many cases and rheumatoid arthritis that has become resistant to steroid
therapy may, once again, react favourably to the administration of steroids.
Tuberculosis
Tuberculosis of the spleen is not so uncommon as is sometimes believed. It occurs
chiefly in adults between 20 and 40 years of age. When a patient has splenomegaly with
asthenia, loss of weight and an evening fever, itis well to bear in mind the possibility that
the enlargement of the spleen may be due to tuberculosis. Too often the signs lead to
erroneous diagnosis of leukaemia or some other disorder for which splenectomy is not
indicated. Occasionally tuberculosis of the spleen produces portal hypertension. Another
form is cold abscess, which is very rare. Splenic puncture followed by culture or guinea-
pig inoculation will yield positive results. A therapeutic test with antituberculous drugs
(Chapter 8) brings about some improvement, and there is less danger of dissemination of
the tubercle bacilli if splenectomy is undertaken. The operation, which is usually
rendered difficult because of adhesions, is contraindicated only if other active tuberculous
lesions are found to be present. Otherwise, the results of splenectomy in the treatment of
tuberculosis of the spleen are excellent.
Neoplasms
The most common benign tumour of the spleen is the haemangioma which may, on
occasion, develop into a haemangiosarcoma. Splenectomy may be necessary. The
commonest cause of neoplastic enlargement of the spleen is lymphoma. Splenectomy
may play a part in the management of these conditions (Chapter 17), but the role is now
limited.
Porphyria
Porphyria is a hereditary error of catabolism of haemoglobin in which porphyrinuria
occurs. The abdominal crises, which are characterised by violent intestinal colic with
constipation, are liable to be precipitated by the administration of barbiturates, for which
these patients have an idiosyncrasy. The patient is anaemic, frequently suffers from
photosensitivity and, in advanced stages of the disease, neurological or mental symptoms
(from damage to the brain) are commonly present. On examination, the spleen will be
found to be enlarged. On a number of occasions the splenic enlargement, which is usually
well marked, has been overlooked and the abdomen has been opened on the presumptive
diagnosis of intestinal or appendicular colic, with negative findings. Another
manifestation of acute porphyria is spasmodic abdominal pain followed by jaundice.
Two methods of establishing the diagnosis are available.
• The urine is sometimes normal in colour —usually itis orange (often dismissed as
‘concentrated’). If a urine
specimen is left exposed to daylight for a few hours it develops a port wine colour,
particularly near the surface where itis exposed to the air. There are several conclusive
laboratory tests for porphyrinuria.
• Radiography of the abdomen —serial radiographs show areas of intestinal spasm
causing short segments of gaseous dilatation of the small and large intestine, and
especially of the caecum.
Treatment
Often there is a striking decrease in the serum sodium level and the patient is improved
considerably by the infusion of isotonic saline solution with careful control of electrolyte
balance. Methadone is the best drug to relieve the abdominal pain. If a sedative is
required, one of the phenothiazines (e.g. chlorpromazine) should be given. Splenectomy
is not of value except in the uncommon erythropoeitic type with splenomegaly.
Gaucher’s disease
As mentioned, the spleen may take an active part in the storage of abnormal lipoids, as
does the remainder of the reticuloendothelial system. In the case of Gaucher’s disease the
lipoid in question is glucocerebroside. Gaucher’s disease, which is rare, is characterised
by enormous enlargement of the spleen, which may weigh 8 or 9 lbs (3.6—4.1 kg). In
most cases the splenic enlargement begins in early childhood, often before the age of 12
years, although the patient rarely seeks advice before adult life. Until the splenic
enlargement becomes massive the symptoms are few. There is anaemia, a yellowish-
brown discoloration of the skin of the hands and face, and a curious conjunctival
thickening (pinguecula) that helps to clinch the clinical diagnosis. Slavonic and Jewish
races appear to be more prone to the disease than other races. The diagnosis is confirmed
by finding Gaucher’s cells in the bone marrow.
Treatment
Splenectomy rids the patient of a large abdominal swelling, but the operation is difficult
because of perisplenitis and friability of the splenic pulp. It does not greatly influence the
course of the disease, but because it reduces the hypersplenism (anaemia, leucopenia and
thrombocytopenia) and makes the patient more comfortable, the procedure may be
indicated.
Leukaemia
Leukaemia is one of the conditions to be considered in the differential diagnosis of
splenomegaly. The diagnosis can be made by examination of a blood or marrow film.
The main treatment is chemotherapy or radiotherapy; occasionally marrow
transplantation may be necessary. Splenectomy during the chronic phase of chronic
granulocytic leukaemia will not reduce the incidence of blastic transformation or improve
survival. The procedure should be reserved for hypersplenism occurring during the
chronic phase, or for when bone marrow transplantation might be necessary. In rare
instances the removal of a symptomatic enlarged spleen during the blastic phase produces
relief, but the period of relief is brief and the operation hazardous.
Splenectomy is occasionally indicated for palliation of a painful bulky spleen in chronic
lymphocytic leukaemia, but only after consultation with an experienced haematologist.
Abscess of the spleen
If a splenic embolus is infected, and the primary condition does not prove fatal, a splenic
abscess may be expected to follow Other sources of metastatic abscess of the spleen are
typhoid and paratyphoid fever, osteomyelitis, otitis media and puerperal sepsis. An
abscess in the right upper pole of the spleen may rupture and form a left
subdiaphragmatic abscess. If the abscess is in the lower pole, rupture results in diffuse
peritonitis.
Treatment
As a rule, owing to dense adhesions, drainage of the abscess is the only course. Very
rarely, splenectomy may be possible with the abscess in situ. The drainage may be
performed percutaneously, under ultrasonic or CT guidance, so avoiding the need for
operative intervention.