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Splenomegaly – Causes, Symptoms and Treatment

Splenomegaly refers to an abnormal enlargement of the spleen. It is usually a sign of an underlying condition and is diagnosed through imaging.

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Things worth knowing about "Splenomegaly"

Splenomegaly refers to an abnormal enlargement of the spleen. It is usually a sign of an underlying condition and is diagnosed through imaging.

What is Splenomegaly?

Splenomegaly is the medical term for an abnormal enlargement of the spleen beyond its normal size. The spleen is an essential organ of the immune and circulatory systems, located in the upper left abdomen. It filters the blood, stores blood cells, and helps fight pathogens. In adults, a spleen measuring more than 12 cm along its long axis is considered enlarged. An exceptionally large spleen is referred to as massive splenomegaly.

Causes

Splenomegaly is generally not a disease in itself but rather a sign of an underlying condition. The most common causes can be grouped into several categories:

Infectious Diseases

Viral infections: e.g., Epstein-Barr virus (infectious mononucleosis), cytomegalovirus, HIV
Bacterial infections: e.g., sepsis, typhoid fever, bacterial endocarditis
Parasitic infections: e.g., malaria, leishmaniasis, toxoplasmosis

Hematological Disorders

Leukemias (e.g., chronic myeloid leukemia)
Lymphomas (e.g., Hodgkin lymphoma, non-Hodgkin lymphoma)
Hemolytic anemias (e.g., sickle cell disease, hereditary spherocytosis)
Myelofibrosis

Liver and Portal Venous Disorders

Liver cirrhosis with portal hypertension (increased pressure in the portal venous system)
Portal vein thrombosis
Right-sided heart failure (venous congestion)

Immunological and Inflammatory Conditions

Systemic lupus erythematosus (SLE)
Rheumatoid arthritis (Felty syndrome)
Sarcoidosis

Metabolic Disorders

Gaucher disease
Niemann-Pick disease
Amyloidosis

Symptoms

Mild splenomegaly often causes no symptoms and may be discovered incidentally. With more pronounced enlargement, the following symptoms may occur:

Pressure or fullness in the upper left abdomen, especially after eating
Pain in the left upper abdomen, which may radiate to the left shoulder
Early satiety when eating (due to the stomach being displaced)
General fatigue and weakness
Signs of hypersplenism: anemia, increased susceptibility to infections (leukopenia), and a tendency to bleed (thrombocytopenia) due to excessive destruction of blood cells in the enlarged spleen

In rare cases, a severely enlarged spleen can rupture spontaneously or following trauma, which constitutes a life-threatening emergency.

Diagnosis

The diagnosis of splenomegaly is established through various examinations:

Physical examination: Palpation and percussion of the abdomen. A palpable spleen is usually significantly enlarged.
Ultrasound (sonography): The standard method for measuring spleen size and assessing its structure.
CT or MRI: Used for inconclusive findings or detailed diagnostics, such as ruling out tumors or cysts.
Blood tests: Complete blood count, liver enzymes, inflammatory markers, and serological tests for infectious diseases.
Bone marrow biopsy: Performed when a hematological disorder is suspected.

Treatment

Treatment of splenomegaly is always directed at the underlying cause:

Infections: Antiviral, antibiotic, or antiparasitic therapy
Hematological disorders: Chemotherapy, radiation therapy, targeted medications, or stem cell transplantation
Portal hypertension: Treatment of the liver disease; in some cases, placement of a TIPS (transjugular intrahepatic portosystemic shunt)
Hypersplenism: In severe cases, surgical removal of the spleen (splenectomy) may be necessary. Vaccination against encapsulated bacteria (e.g., pneumococci, meningococci, Haemophilus influenzae) should be administered beforehand.

Patients with an enlarged spleen should avoid contact sports and activities with a high risk of injury, as the spleen is more susceptible to rupture.

References

Longo DL, Fauci AS et al. - Harrison's Principles of Internal Medicine, 21st Edition. McGraw-Hill Education, 2022.
Wilkins BS - The spleen. In: Underwood's Pathology, 7th Edition. Elsevier, 2019.
Pozo AL, Godfrey EM, Bowles KM - Splenomegaly: investigation, diagnosis and management. Blood Reviews, 23(3):105-111, 2009. PubMed PMID: 19345465.

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