Splenomegaly generally denotes a palpably enlarged spleen. However, it may also refer to an enlarged spleen detected by an imaging test. It is not uncommon for a radiologist interpreting a chest x-ray to comment that the spleen seems enlarged (usually considered an incidental finding). Splenomegaly can be found in 3% of the general population.
An average human spleen is 10 cm long and weighs 150 grams. It may be difficult to palpate an enlarged spleen in the settings of obesity, a muscular abdominal wall, or the inability to sufficiently relax the abdominal musculature. In these cases, spleen size may need to be determined by imaging.
Approach to splenomegaly
It is difficult to create a stepwise algorithmic approach to the patient with splenomegaly. Urgent presentations include sudden pain associated with splenomegaly, splenic sequestration crisis of sickle cell anaemia, and splenic vein or portal vein thrombosis. Otherwise presentation is often incidental on physical examination or imaging. Categorisation by presentations such as massive splenomegaly, isolated splenomegaly, or accompanying symptoms could be considered. However, these categories encompass diverse diagnoses. For example, thalassaemia, chronic myeloid leukaemia, Gaucher's disease, hairy cell leukaemia, myelofibrosis, and malaria may all present with a markedly enlarged spleen. Isolated splenomegaly is a feature of diagnoses such as splenic marginal lymphoma or benign splenic neoplasms. Accompanying symptoms are non-specific; for example, fever may occur with lymphomas, malaria, endocarditis, or infectious mononucleosis.
- Alcohol induced
- Hepatic steatosis
- Primary biliary cholangitis (PBC)
- Primary sclerosing cholangitis
- Hodgkin's lymphoma
- Non-Hodgkin's lymphoma (NHL)
- Waldenström's macroglobulinaemia or lymphoplasmacytic lymphoma
- Acute myeloid leukaemia (AML)
- Chronic myeloid leukaemia (CML)
- Acute lymphocytic leukaemia (ALL)
- Chronic lymphocytic leukaemia (CLL)
- Hairy cell leukaemia
- Polycythaemia vera
- Essential thrombocytosis
- Splenic metastases
- Autoimmune haemolytic anaemia
- Rheumatoid arthritis (RA)
- Felty syndrome
- Systemic lupus erythematosus
- Epstein-Barr virus (EBV)
- Sepsis-related splenic abscesses
- Chronic hepatitis C
- Chronic hepatitis B
- Sickle cell anaemia
- Cytoskeletal defects
- Benign splenic tumours
- Portal vein thrombosis
- Splenic vein thrombosis
- Budd-Chiari syndrome
- Haemophagocytic lymphohistiocytosis (HLH)
- Gaucher's or Niemann-Pick disease
- Severe dengue
- Splenic rupture
- Subcapsular haemorrhage
- Secondary to granulocyte colony-stimulating factor (G-CSF)
Terri L. Parker, MD
Assistant Professor of Medicine
Section of Hematology
Yale School of Medicine
Smilow Cancer Center at Yale-New Haven
TLP declares that she has no competing interests.
Dr Terri L. Parker would like to gratefully acknowledge Dr Alan E. Lichtin, the previous contributor to this monograph.
AEL has received research support from Amgen.
Priyanka Mehta, MD
Bristol Haematology and Oncology Centre
PM declares that she has no competing interests.
Ruben A. Mesa, MD
Professor of Medicine
RAM declares that he has no competing interests.
Giovanni Barosi, MD
Director of the Laboratory of Clinical Epidemiology
IRCCS Policlinico S. Matteo Foundation
GB declares that he has no competing interests.
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