What is splenomegaly?

Splenomegaly is an enlargement of the spleen beyond its normal size. The spleen is an organ involved in the production and maintenance of red blood cells, the production of certain circulating white blood cells, and is a part of the lymph system and the immune system. Because of its wide variety of functions, the spleen may be affected by many conditions involving the blood or lymph system, and by infection, cancers, liver disease, and parasites.

What are the causes?

1. Infections
Parasitic infections
Viral infections
Bacterial infections

2. Diseases involving the liver
Cirrhosis (portal vein obstruction, portal hypertension)
Sclerosing cholangitis
Wilson’s disease
Biliary atresia
Cystic fibrosis

3. Haemolytic anaemias
Thalassaemia
Haemoglobinopathies
Haemolytic anaemia due to G6PD deficiency
Immune haemolytic anaemia

4. Cancer
Leukaemia
Lymphoma
Hodgkin’s disease

5. Other causes
Sarcoidosis

What is the diagnosis?

Splenomegaly is usually detected during a physical examination by a health care provider. A detailed examination of the abdomen will probably be performed. Diagnostic tests may be required, including:

1. Blood tests such as a CBC
2. Tests for suspected causes
3. Abdominal film or CT scan

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

It moves with respiration.
It 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 - the 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.

Investigations
Investigation will depend on the specific clinical presentation of the patient but may include:
Abdominal ultrasound imaging, MRI scanning, CT scanning.
FBC, reticulocytes, blood film.
Haemoglobinopathy screen.
LFTs.
Virology, microbiology.
Serum protein electrophoresis.
Peripheral blood cell markers (leukaemia, lymphoma).
Radioisotope liver and spleen scan.1
Liver biopsy, bone marrow biopsy, lymph node biopsy.

Splenomegaly in children
This is most commonly caused by infection, autoimmune disorders or haemolysis. It may be a presenting feature of neoplasia (e.g. metastases neuroblastoma).

Causes include:
Infection: glandular fever, cytomegalovirus (CMV), other viral infections, often accompanied by lymphadenopathy; bacterial, protozoan, and fungal infections.
Autoimmune: juvenile rheumatoid arthritis.
Haemolysis: hereditary spherocytosis, sickle cell anemia, thalassaemia.
Neoplasia: acute lymphoblastic leukaemia (ALL), Hodgkin's disease and Non-Hodgkin's lymphoma (NHL), acute or chronic myeloblastic leukaemia, neuroblastoma.
Inherited diseases: Gaucher's disease and other storage disorders.

Hypersplenism
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 reticular-endothelial 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.

Management
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 Splenectomy, Hyposplenism and Asplenia).

 

Select Language English Russian French German Italian Japanese Korean Portuguese Spanish Greek Dutch Hindi