What are the other Names for this Condition? (Also known as/Synonyms)

• Enlarged Spleen
• Spleen Swelling
• Splenic Enlargement

What is Splenomegaly? (Definition/Background Information)

• The spleen is a small organ situated just below the rib cage, on the left side of the body. It forms a part of the lymph system, which helps filter blood and maintains proper levels of different blood cell types. When there is an enlargement of this organ, the condition is called Splenomegaly
• It can develop owing to several medical conditions, including infections, liver diseases, blood disorders, cancer, and certain autoimmune diseases. Individuals of African-American and Ashkenazi Jewish descent tend to have a higher risk for Splenomegaly
• Some typical symptoms of Splenomegaly are feeling full after eating little, constant hiccups, and abdominal pain. The condition can increase an individual’s susceptibility to infections; it can also result in severe complication, such as a ruptured spleen
• The condition is treated by a surgical removal of the spleen, when warranted. Generally, if no underlying cause is apparent and symptoms are absent, a healthcare provider will assess and monitor the Enlarged Spleen, before deciding to remove the organ. Once the spleen is removed, vaccinations may be necessary to protect against certain infectious conditions such as meningitis and pneumonia
• The prognosis for Splenomegaly is dictated by whether it is caused by an acute infection or a chronic disease. If an infection is diagnosed and treated promptly, the outcome is generally good. However, Enlarged Spleen owing to a chronic condition usually lead to poorer outcomes
• Seeking medical attention for infections that could lead to Splenomegaly is one way of preventing or minimizing its occurrence. Avoiding injury to the spleen, during participation in sports or from automobile accidents (by wearing seat belts), is also an important consideration in reducing the risk for Splenomegaly

Who gets Splenomegaly? (Age and Sex Distribution)

• Splenomegaly can occur in individuals of all ages and both male and female genders
• However, individuals with African-American or Ashkenazi ancestry are reportedly more prone to the condition

What are the Risk Factors for Splenomegaly? (Predisposing Factors)

The risk factors associated with Splenomegaly include:

• Individuals affected with sickle cell and hemolytic anemia, especially prevalent in African-American population
• Individuals affected with Niemann Pick disease and Gaucher disease, especially prevalent in people of Ashkenazi-Jewish descent
• Children and young adults affected with severe infections, such as mononucleosis
• Residing in malaria-endemic regions; traveling to areas where malaria is widespread
• Those with certain chronic conditions, such as rheumatoid arthritis or lupus, are more susceptible to Enlarged Spleen

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Splenomegaly? (Etiology)

Splenomegaly may be caused by a variety of factors that include the following:

• Infections, such as:
  • Bacterial infections (including cat scratch disease)
  • Viral infections such as mononucleosis
  • Parasitic infections such as malaria
• Liver diseases:
  • Biliary atresia: Blockage in the tube that carries bile liquid from the liver to gall bladder
  • Cirrhosis or scarring of the liver with resultant poor liver function
  • Cystic fibrosis resulting in the building-up of thick, sticky mucus in the lungs, digestive tract and other organs
  • Portal hypertension: High blood pressure in the portal vein system
  • Portal vein obstruction: A condition where the portal hepatic vein is blocked/affected
  • Sclerosing cholangitis: A condition marked by inflammation in the bile ducts, inside and outside the liver
• Blood diseases:
  • Hemoglobinopathies: Group of blood disorders affecting the red blood cells
  • Hemolytic anemia or the abnormal breakdown of red blood cells
  • Idiopathic autoimmune hemolytic anemia: A condition that manifests as a marked decrease in the red blood cell count
  • Immune hemolytic anemia: Premature destruction of circulating red blood cells
  • Myelodysplastic syndrome: In this condition, damage to the blood cells and their precursors in the bone marrow occur
  • Polycythemia vera: A bone marrow disease leading to abnormal increase in the number of red blood cells
  • Thalassemia: A condition in which the amount of healthy red blood cells and hemoglobin is low
• Cancers, such as:
  • Leukemia, or cancer of blood or bone marrow
  • Hodgkin’s disease or cancer of lymphocytes
• Felty syndrome: It is a rare disorder comprising of rheumatoid arthritis, enlargement of the spleen, decreased white blood cell count, and repeat infections
• Long-term autoimmune disorders such as systemic lupus erythematosus and rheumatoid arthritis
• Sarcoidosis: An inflammatory condition of the lymph nodes and other organs in the body
• Sickle cell splenic crisis: A condition that occurs in sickle cell disease, when there is a sudden breakdown of red blood cells

What are the Signs and Symptoms of Splenomegaly?

The signs and symptoms of Splenomegaly may include:

• Hiccups, which are frequent and causing discomfort
• Inability to eat large meals
• Abdominal pain
• Enlarged Spleen upon visual abdominal examination

How is Splenomegaly Diagnosed?

A healthcare provider may employ many of the following methods to diagnose Splenomegaly:

• A thorough physical examination and assessment of medical history
• Blood tests to check the number of different blood cell types (complete blood counts)
• Imaging tests such as ultrasound scan of the abdomen
• Computerized tomography (CT) scan of the abdomen to check for the size of spleen
• Abdominal magnetic resonance imaging (MRI) scan to check flow of blood through spleen
• Liver function tests (LFT)
• Blood cultures
• Hemoglobin electrophoretic studies
• Bone marrow culture and bone marrow biopsy
• In extremely rare cases, examination of the spleen after surgical removal of the organ to check for abnormalities

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Splenomegaly?

Some possible complications associated with Splenomegaly may include:

• Reduction in the number of red blood cells, white blood cells, and platelet counts in the body leading to infections, anemia, and increased bleeding
• Ruptured spleen: A ruptured spleen can lead to life-threatening bleeding inside the abdominal cavity

How is Splenomegaly Treated?

The treatment methods for Splenomegaly may involve the following:

• In cases where the individual has an Enlarged Spleen without symptoms, a healthcare professional may opt to wait several months, before deciding on a course of action
• Surgical removal of spleen, through a splenectomy, in very serious and critical cases
• Once a decision to remove the spleen is made, the individual is advised to take certain vaccinations, both before and after the surgery, to protect him/her from infections. The recommended vaccinations may include:
  • Pneumococcal vaccine
  • Meningococcal vaccine
  • Haemophilus influenzae type B vaccine
• It is also advised that the individual get a pneumococcal vaccine, once every 5 years, following the removal of spleen
• Antibiotics, such as penicillin, may be prescribed to combat infections post-surgery
• Treatment of the underlying condition depending on the cause

How can Splenomegaly be Prevented?

The following measures can help reduce the risk of Splenomegaly:

• Considering adequate safety measures and use of protective sports gear prior to participation in contact sports, such as soccer and football, to minimize the risk of injuring the spleen
• Avoiding contact sports in case of an infected spleen
• Wearing seat belts, while traveling in vehicles
• Getting vaccinated on time, so that infections that may result in Splenomegaly may be minimized/eliminated
• Undertaking regular visits to a healthcare provider, if there is an increased risk for Splenic Enlargement

What is the Prognosis of Splenomegaly? (Outcomes/Resolutions)

The prognosis of Splenomegaly generally depends on the disease process and treatment required.

• When caused by an acute infection, it can be diagnosed and treated promptly, and the outcome is generally good
• When Splenomegaly is caused by chronic conditions, the outcome generally depends on the severity of the underlying condition
• Ruptured spleen is a medical emergency; in such cases, without prompt treatment, the prognosis is guarded
• The prognosis for recovery following surgical removal of the spleen is generally good in the majority of cases

Additional and Relevant Useful Information for Splenomegaly:

The following DoveMed link will help you understand splenectomy, the surgical procedure to remove spleen:

http://www.dovemed.com/common-procedures/procedures-surgical/splenectomy/