Infective

Most infective causes present with fever, malaise and anorexia. With typhus, there will be a history of foreign travel. Typhus is conveyed between hosts by arthropods. The patient complains of severe constitutional symptoms with headache, vomiting, photophobia and toxaemia. With typhoid, there will usually be a history of foreign travel or it will occur in the immigrant population. There will be a history of malaise, headache, fever, cough, constipation initially and then diarrhoea. Epistaxis and abdominal pain may occur. TB usually presents with weight loss, night sweats and a cough. If septicaemia is responsible, there will usually be an obvious cause, and the patient may already be hospitalised. With splenic abscess, there may be a history of endocarditis, lung abscess, drug abuse or it may occur in an immunocompromised host. Syphilis is rare and there may be a history of contact. With leptospirosis, there is often a history of contact with rats, particularly when swimming in rivers where there are rats by the riverside. Fever, jaundice, headache, haemoptysis and haematuria are presenting symptoms. With malaria, there is usually a history of travel to an area where it is endemic. The patient usually presents within two months of travel abroad with malaise, myalgia, sweating, coldness, followed by rigors, high fever and drenching sweats. Hydatid disease occurs most frequently in rural sheep-farming regions, e.g. Wales. The commonest presentation is with a cyst in the lung causing dyspnoea, haemoptysis or anaphylaxis.

Inflammatory

Rheumatoid arthritis may be apparent. Sarcoidosis may present with lymphadenopathy, fever and malaise, as well as hepatosplenomegaly. Lupus may present with protean manifestations with cutaneous, musculoskeletal, renal, pulmonary and haematological problems. Amyloid may be primary or secondary. There may be cutaneous, cerebral, cardiac or endocrine manifestations. With secondary amyloidosis there may be a history of chronic infection, e.g. TB or inflammatory disease, e.g. rheumatoid arthritis. There may also be a history of neoplasis. Secondary amyloidosis usually affects the kidney and liver as well as the spleen.

Neoplastic

There will often be generalised signs of malignancy. Often there will be general malaise with weight loss, and with lymphoma, there will be a history of night sweats. There may also be a tendency to spontaneous bruising. Primary tumours of the spleen and splenic metastases are rare.

Haemolytic disease

Haemolytic disease usually presents with the symptoms of anaemia, namely tiredness and malaise. Jaundice may be present, although is often not very deep, merely giving a lemonish tinge to the skin. With excessive breakdown of red blood cells, pigment stones may form in the gall bladder and the disease may present with acute cholecystitis.

Storage diseases

Apart from variable hepatosplenomegaly, these may present with neurological problems, skeletal deformities or mental deterioration.

Deficiency diseases

There may be a history of pernicious anaemia. Splenomegaly is usually mild. With iron-deficiency anaemia, there may be dietary deficiency, malabsorption or blood loss. Check for a history of blood loss.

Splenic vein hypertension

There may be an obvious history, with cirrhosis and signs of liver failure. Isolated splenic vein thrombosis is rare.

Non-parasitic cysts

These are rare but may arise from organised haematomas, infarcts or inflammation. They are usually isolated findings when the patient is being investigated for other conditions.

General

The spleen appears from below the tip of the left tenth rib and enlarges towards the umbilicus. It is firm and smooth and usually has a notch on its upper edge. It moves with respiration, is dull to percussion and it is impossible to get above it.

Infective

With typhus, there is only moderate splenomegaly. The patient will often be toxic with a generalised maculopapular rash. With typhoid, there is moderate splenomegaly, the patient being pyrexial with a tender abdomen and with rose spots on the trunk. Again with TB, the splenomegaly is only moderate. There may be chest signs. With septicaemia, there is usually an obvious cause and again there is only moderate splenomegaly. With splenic abscess, again there is only moderate splenomegaly and the spleen may be tender and feel irregular. With glandular fever, the patient will be pyrexial with lymphadenopathy, and occasionally there is a rash, especially if the patient has been given ampicillin. Splenomegaly is moderate. With syphilis, there may be fever, malaise, lymphadenopathy and a rash on the trunk, palms and soles. Rarely, there may be hepatitis, meningism and uveitis. With leptospirosis ictohaemorrhagica, the patient is usually ill with pyrexia, jaundice and purpuric rash. There may be signs of meningitis. There also may be tender muscles (myositis). There may be signs of acute renal failure. With malaria, there is often anaemia, jaundice and hepatosplenomegaly. With hydatid disease, the liver is usually more affected than the spleen. There may be hepatomegaly in addition to splenomegaly.

Inflammatory

With inflammatory disease, the spleen is rarely grossly enlarged. Changes of rheumatoid arthritis may be apparent elsewhere in the body. With sarcoidosis, there may be lymphadenopathy as well as hepatosplenomegaly. With lupus, there may be cutaneous manifestations as well as musculoskeletal, renal and pulmonary manifestations. With amyloid, in addition to hepatosplenomegaly, there may be signs of carpal tunnel syndrome, peripheral neuropathy, purpura and a large tongue.

Neoplastic

There may be massive splenomegaly associated with chronic myeloid leukaemia, myelofibrosis and lymphoma. Other signs such as lymphadenopathy and spontaneous bruising may be present.

Haemolytic disease

The patient may have a mild jaundice together with moderate splenomegaly. A purpuric rash may be apparent with thrombocytopenic purpura.

Storage diseases

These are rare. There may be signs of cardiac failure, skeletal deformity or neurological deficits.

Splenic vein hypertension

Signs of liver failure, e.g. spider naevi, liver palms, caput medusae and jaundice may be present.

Non-parasitic cysts

The only finding may be splenomegaly.

■ FBC, ESR, blood film
Hb ↓ anaemias. Hb ↑ polycythaemia. WCC ↑ infection, leukaemia, abscess. ESR ↑ infection, malignancy. WCC ↓, platelets ↓ hypersplenism. PCV ↑ polycythaemia. MCV ↑ pernicious anaemia. Blood film – spherocytosis, malaria.

■ U&Es
Urea ↑, creatinine ↑ renal failure, e.g. with leptospirosis, amyloid, septicaemia.

■ LFTs
Cirrhosis

■ Rheumatoid factor
Rheumatoid arthritis.

■ Paul–Bunnell test
Glandular fever.

■ VDRL
Syphilis.

■ Serum iron and serum ferritin
↓ iron-deficiency anaemia.

■ Parietal cell antibodies
Pernicious anaemia.

■ Serum vitamin B₁₂
Pernicious anaemia.

■ Blood culture
Septicaemia. Leptospirosis. Typhoid.

■ Stool culture
Typhoid.

■ Urine culture
Typhoid. Leptospirosis.

■ CSF culture
Leptospirosis.

■ Complement fixation test
Leptospirosis.

■ ELISA/immunofluorescence
Typhus.

■ Bone marrow aspirate
Myelofibrosis. Polycythaemia. Leukaemia. Gaucher’s disease.

■ US
Cause of splenomegaly, e.g. cyst, tumour, diffuse enlargement. Cirrhosis of the liver.

■ CT
Causes of splenomegaly, e.g. cyst, tumour, diffuse enlargement, cirrhosis.