Splenomegaly: Symptoms, Causes

Brief overview

  • Symptoms:Pain in the upper abdomen to severe pain in ruptured spleen.
  • Causes and risk factors: Infectious diseases, hereditary diseases, cancers, metabolic diseases and others.
  • Diagnosis: medical history, palpation of the spleen, ultrasound examination, analysis of blood values, further examinations
  • Treatment: Depending on the underlying disease, in some cases surgical removal of the spleen.

What is splenomegaly?

An enlarged spleen is a common symptom. It occurs in various diseases. These include infectious diseases, hereditary diseases, diseases of the blood or liver, and many others.

One of its tasks is to capture and break down old and deformed blood cells as well as microorganisms found in the blood. In addition, immune cells mature in it. It is possible to live without a spleen. However, the risk of serious infections then increases.

What are the symptoms?

Symptoms of the underlying disease in splenomegaly

Many different diseases lead to splenomegaly, among others. Depending on this underlying disease, patients have symptoms. The physician bases his diagnosis on the following correlations, among others:

  • In infectious diseases: Fever, fatigue, swelling of the lymph nodes.
  • In malignant cancer diseases and similar: Weight loss, night sweats, fever
  • In hematopoietic disorders: Fatigue, weakness, pallor

Symptoms causing splenomegaly

An abnormal swelling of the spleen is usually palpable under the left costal arch. It may cause pain, for example, if it presses on nerves or displaces other organs. If the spleen swells too much for the capsule that surrounds it, it is possible for it to rupture. The so-called rupture of the spleen is accompanied by severe pain in the left upper abdomen. In many cases, this pain radiates to the left shoulder.

The causes that may lead to splenomegaly are manifold. They can be divided into several groups.

Blood diseases

There are benign and malignant diseases of the blood that cause splenomegaly. Benign ones include congenital defects of red blood cells.

These include:

  • Sickle cell disease
  • Thalassemia
  • Hereditary spherocytosis
  • Glucose-6-phosphate dehydrogenase deficiency

Malignant diseases of the blood that cause an enlarged spleen include leukemias and lymphomas, as well as myeloproliferative disorders such as osteomyelofibrosis or juvenile myelomonocytic leukemia, which are various forms of blood cancers.

Infections

  • Bacterial sepsis
  • Leishmaniasis
  • Malaria
  • Syphilis
  • Typhoid fever
  • Tuberculosis
  • Echinococcosis

Portal vein damage

If there is an outflow obstruction in the portal vein, the blood backs up into the spleen (congestive spleen). Reasons for this include:

  • Heart failure
  • Liver cirrhosis or fibrosis (in this case, there is usually an enlarged liver in addition to an enlarged spleen)
  • Portal vein thrombosis
  • Budd-Chiari syndrome

Storage diseases

  • Glycogen storage disease
  • Niemann-Pick disease
  • Gaucher disease
  • Mucopolysaccharidoses

Also in these cases, splenomegaly often persists for years as a chronic symptom.

Immunological diseases

Various immunological diseases are possible causes of splenomegaly, which is then also usually chronic. These include:

  • Chediak-Higashi syndrome
  • Kawasaki syndrome
  • Histiocytoses
  • Chronic granulomatosis
  • Autoimmune lymphoproliferative syndrome (ALPS)

Other possible causes

In rare cases, splenic swelling occurs in collagenoses such as systemic lupus erythematosus, Still’s disease, or juvenile rheumatoid arthritis. Splenomegaly is also possible in sarcoidosis.

Connections between an enlarged spleen and stress or “unhealthy” lifestyle are described in the field of alternative medicine, but have not been proven by conventional medicine or science.

Examinations and diagnosis

  • Have you suffered from an infection lately?
  • Are you suffering from a chronic or malignant disease?
  • Do you have a fever?
  • Have you lost weight unintentionally lately?
  • Do you wake up at night drenched in sweat?

Physical examination

If your doctor palpates it, splenomegaly is present. He then confirms this finding in an ultrasound examination by measuring the spleen. In addition, the ultrasound may show evidence of liver damage or disease of the portal vein.

Further diagnostics

Once your doctor has diagnosed splenomegaly, further tests are necessary to find the causes of the enlarged spleen. First, he usually takes blood from the patient for analysis in the laboratory. There, they examine:

  • Blood count and blood smear (number of red blood cells, white blood cells, and platelets, including a listing of the different types of white blood cells and number of young red blood cells).
  • Indications of liver injury: Transaminases (ALAT, ASAT), bilirubin.
  • Immune parameters: C-reactive protein, antinuclear antibodies, rheumatoid factors, Coombs test, electrophoresis.
  • Signs of viral infections

The physician then usually initiates further diagnostic steps, such as a chest X-ray, computed tomography of the abdomen, or a bone marrow biopsy.

Treatment of splenomegaly

Splenomegaly is usually a symptom of another underlying disease. Once the underlying disease is diagnosed, it is treated. With effective therapy, splenomegaly often disappears.

This is known as overwhelming post-splenectomy infection (OPSI). The immune system then often no longer fights off encapsulated bacteria such as pneumococci or meningococci sufficiently well.

Course of disease and prognosis

Another complication that often results in splenectomy is hypersplenism. It represents an overfunction of the spleen. It then removes more blood cells than necessary (excessive phagocytosis).