Splenomegaly and Hypersplenism

The spleen is involved in producing protective humoral antibodies, the production and maturation of B and T cells and plasma cells, removal of unwanted particulate matter (e.g. bacteria) and also acts as a reservoir for blood cells, especially white cells and platelets. When the spleen is palpable it has usually reached at least twice its normal size.

• Left upper quadrant (LUQ) mass or "uncomfortable" abdominal pain, early satiety from compressed stomach.
• Pancytopenia due to hypersplenism (see below).

Examination

When considering whether a LUQ mass is an enlarged spleen, features of an enlarged spleen include:

• Moves with respiration
• Enlarges towards the right iliac fossa (RIF) - always start palpation in the RIF and move across towards the right upper quadrant (or a massive splenomegaly may be missed)
• You cannot palpate above it - upper margin lies under the ribs
• You may feel a notch
• It is dull to percussion
• Always remember to check for any accompanying lymphadenopathy and/or features of liver disease.

Investigation will depend on the specific clinical presentation of the patient but may include:
• Abdominal ultrasound imaging, MRI, CT
• Full blood count, reticulocytes, blood film
• Haemoglobinopathy screen
• Liver function tests
• Virology, microbiology
• Serum protein electrophoresis
• Peripheral blood cell markers (leukaemia, lymphoma)
• Radioisotope liver and spleen scan
• Liver biopsy, bone marrow biopsy, lymph node biopsy

• Haematological
  • Haemolytic anaemias (eg Thalassaemia, red cell defects, Sickle cell anaemia)
  • Acute leukaemias, chronic leukaemias
  • Polycythaemia rubra vera
  • Macroglobulinaemia
  • Lymphoma (Hodgkin's disease and non-Hodgkin's lymphoma)
  • Essential thrombocythaemia
  • Myelofibrosis
• Infections
  • Malaria
  • Schistosomiasis
  • Visceral leishmaniasis (Kala-azar)
  • Tuberculosis, brucellosis
  • Glandular fever, viral hepatitis
  • Infective endocarditis
• Tumours and cysts
  • Splenic abscesses
  • Splenic metastases
  • Cysts, e.g. hydatid, dermoid
  • Tumours, e.g. haemangioma
• Congestive splenomegaly
  • Liver cirrhosis
  • Budd Chiari syndrome
  • Portal or splenic vein obstruction
  • Heart failure
• Connective tissue disorders
  • Systemic lupus erythematosus
  • Felty's syndrome
• Other disorders
  • Gaucher's disease
  • Niemann Pick disease
  • Histiocytosis X
  • Amyloidosis

Causes of massive splenomegaly

• Chronic myeloid leukaemia
• Myelofibrosis, malaria (hyper-reactive malarial splenomegaly)
• Leishmaniasis
• 'Tropical splenomegaly' (idiopathic; Africa, SE Asia)
• Gaucher's syndrome

Splenomegaly in children

This is most commonly caused by infection, autoimmune disorders or haemolysis. It may be a presenting feature of neoplasia (e.g. metastatic neuroblastoma). Causes include:

• Infection: Glandular fever, CMV, other viral infections, often accompanied by lymphadenopathy, bacterial, protozoal, and fungal infections.
• Autoimmune: juvenile rheumatoid arthritis
• Haemolysis: hereditary spherocytosis, sickle cell anaemia, Thalassaemia
• Neoplasia: ALL, Hodgkin disease and NHL, acute or chronic myeloblastic leukemia, neuroblastoma.
• Inherited diseases: Gaucher's disease and other storage disorders.

• This is a pancytopenia occurring in patients with an enlarged spleen. It is due to large numbers of cells being pooled and destroyed in the spleen's reticuloendothelial system, and haemodilution because of an increased plasma volume.
• It can present with symptoms of anaemia, infection, or bleeding.
• Bone marrow biopsy shows normal or hyperplastic marrow.
• Splenic sequestration crisis may develop in young children with sickle cell anaemia, which can precipitate hypovolaemic shock and death, and is an indication for splenectomy.

• Treatment of the cause.
• Blood transfusions may be required.
• Open or laparoscopic splenectomy may be indicated to control or stage the disease (e.g. hereditary spherocytosis, Hodgkin's disease).
• Patients with impaired splenic function need prophylactic vaccinations etc. (see separate article on Splenectomy and Hyposplenism).