Splenomegaly

Splenomegaly is a medical term for an enlarged spleen by size or weight ¹. Splenomegaly can be caused by many conditions including infections, liver disease, diseases of the blood or lymphatic system and some cancers ¹. Your spleen is an organ that is a part of your lymphatic system and your immune system. Your spleen filters your blood and maintains healthy red and white blood cells and platelets. The major functions of your spleen include clearance of abnormal red blood cells (erythrocytes), removal of microorganisms and antigens as well as the synthesis of immunoglobulin G (IgG) and white blood cells to fight infections. The spleen also synthesizes the immune system peptides properdin and tuftsin. Also, approximately one-third of circulating platelets are stored in your spleen.

An enlarged spleen or splenomegaly is a symptom of many different conditions, some more serious than others. Your doctor will need to investigate the underlying cause to determine if you need treatment. If an enlarged spleen goes untreated for a long time, it could eventually begin to malfunction. In rare cases, a severely enlarged spleen could rupture, which could cause internal bleeding. In older people, the capsule of the spleen is thin, thus the risk of rupture is higher.

Common causes of enlarged spleen include ¹:

• Infections. Viral infections such as mononucleosis and HIV, bacterial infections such as tuberculosis and endocarditis and parasite infections such as malaria and toxoplasmosis stress the immune function of the spleen. They can cause it to overproduce antibodies and immune cells (hyperplasia).
• Liver disease. Conditions affecting the liver, such as chronic hepatitis or cirrhosis, can cause pressure to build up in the blood vessels that run through the liver and spleen (portal hypertension). Vascular pressure can cause blood to pool and cause your spleen to enlarge.
• Blood cancers. Blood cancers such as leukemia or myeloproliferative neoplasms (MPNs) and lymphomas can infiltrate the spleen with foreign cells that continue to multiply.
• Focal lesions. Benign growths such as a cyst or abscess, as well as metastatic cancer that spreads from somewhere else, can enlarge the spleen.
• Autoimmune diseases. Chronic inflammatory conditions such as systemic lupus erythematosus (SLE), sarcoidosis and rheumatoid arthritis can cause an overactive immune response and spleen hyperplasia ², ³.
• Blood disorders. Conditions such as hemolytic anemia and neutropenia that cause early destruction of red blood cells can overload the spleen, whose job is to remove them.
• Inherited metabolic disorders. Conditions that cause various substances to build up in your blood and store in your organs, such as Niemann-Pick disease, Gaucher disease and sickle cell disease, can infiltrate the spleen.
• Thrombosis. A blood clot that blocks one of the vessels in your liver or spleen can cause pressure and blood to build up in your spleen.

Splenomegaly is a rare condition, with an estimated prevalence of approximately 2% of the total United States population. In adults, there has been no reported predominance in prevalence based on ethnicity, gender, or age. In Asia and Africa, tropical splenomegaly is very common.

An enlarged spleen usually doesn’t cause symptoms. Splenomegaly is often discovered during a routine physical exam. Your spleen is an organ located just below your left rib cage. Your doctor generally can’t feel a normal-sized spleen in adults but can feel an enlarged spleen. Your doctor will likely request imaging and blood tests to help identify the cause.

The size and weight of the spleen may vary and correlate with the weight, height, and sex of an individual, with larger spleen size seen in men compared to women and in heavier or taller individuals. The normal position of the spleen is within the peritoneal cavity in the left upper quadrant adjacent to ribs nine through 12. The normal sized spleen abuts the stomach, colon, and left kidney. A normally sized spleen measures up to 12 cm in craniocaudal length. A length of 12 cm to 20 cm indicates splenomegaly, and a length greater than 20 cm is definitive of massive splenomegaly. The normal weight of the adult spleen is 70 g to 200 g, spleen weight of 400 g to 500 g indicates splenomegaly and spleen weight greater than 1000 g is definitive of massive splenomegaly. The normal sized spleen is usually not palpable in adults. However, it may be palpable due to variations in body habitus and chest wall anatomy.

Splenomegaly may be diagnosed clinically or radiographically using ultrasound, CT imaging, or MRI.

Splenomegaly may be a transient condition due to acute illness or may be due to serious underlying acute or chronic pathology ⁴, ⁵, ⁶. Treatment for splenomegaly focuses on the underlying condition that’s causing it ⁴. Surgically removing an enlarged spleen isn’t usually the first treatment, but is sometimes recommended.

How your spleen works

Your spleen is tucked under your rib cage next to your stomach on the left side of your abdomen. It’s a soft, spongy organ that performs several critical jobs. Your spleen:

• Filters out and destroys old, damaged blood cells
• Prevents infection by producing white blood cells (lymphocytes) and acting as a first line of defense against disease-causing organisms
• Stores red blood cells and platelets, which help your blood clot

An enlarged spleen affects each of these vital functions. As your spleen grows larger, it filters normal red blood cells as well as abnormal ones, reducing the number of healthy cells in your bloodstream. It also traps too many platelets.

Excess red blood cells and platelets eventually can clog your spleen and affect normal functioning. An enlarged spleen may even outgrow its own blood supply, which can damage or destroy sections of the organ.

What is a spleen?

The spleen is the largest lymphoid organ and has a crucial function in the immune system. The spleen is responsible for the production and maturation of IgM, B lymphocytes, and opsonins ⁷. The spleen most importantly protects against infections from polysaccharide-encapsulated bacteria including Streptococcus pneumoniae, Haemophilus influenzae type B, Neisseria meningitidis, Escherichia coli, Salmonella, Klebsiella, and group B Streptococci (SHiNE SKiS) ⁷. The spleen also acts as the primary reservoir for platelets and as a filter for red blood cells (RBCs), removing damaged or malformed red blood cells from the circulation. In addition, the spleen performs extramedullary hematopoiesis.

The normal position of the spleen is within the peritoneal cavity in the left upper quadrant of the abdominal cavity adjacent to ribs nine through 12, just beneath the left diaphragm. The spleen lies behind and to the left side of your stomach (see Figure 1). The normal sized spleen abuts the stomach, colon, and left kidney The spleen resembles a large lymph node and is subdivided into lobules (see Figure 3). However, unlike the lymphatic sinuses of a lymph node, the spaces in the spleen, called venous sinuses, are filled with blood instead of lymph.

A normal spleen ranges in length from 6 to 13 cm and in weight from 75 to 200 g ⁸. The spleen is not normally palpable except in slender young adults ⁸. When the spleen can be felt below the left costal (rib) margin, at rest or on inspiration, spleen enlargement should be assumed and the explanation sought. Although the normal-size or even the abnormally small, spleen can be involved in pathologic processes, with the exception of rubs associated with splenic infarcts, physical examination is generally not helpful in identifying the problem. Nevertheless, the enlarged and palpable spleen is an important clue to the presence of a variety of illnesses ⁸.

Figure 1. Spleen location

Figure 2. Spleen and the lymphatic system

What does the spleen do?

The spleen plays a significant role in hematopoiesis (blood cell production) and immunosurveillance. The major functions of the spleen include clearance of abnormal erythrocytes, removal of microorganisms and antigens as well as the synthesis of immunoglobulin G (IgG). The spleen also synthesizes the immune system peptides properdin and tuftsin ⁹. Also, approximately one-third of circulating platelets are stored in the spleen.

The tissues within splenic lobules are of two types (see Figure 3). The white pulp is distributed throughout the spleen in tiny islands. This tissue is composed of splenic nodules, which are similar to the lymphatic nodules in lymph nodes and are packed with lymphocytes (T lymphocyte cells and B lymphocyte cells). The red pulp, which fills the remaining spaces of the lobules, surrounds the venous sinuses. This pulp contains numerous red blood cells, which impart its color, plus many lymphocytes and macrophages.

Without an immune system, a human being would be just as exposed to the harmful influences of pathogens or other substances from the outside environment as to changes harmful to health happening inside of the body. The main tasks of the body’s immune system are:

• Neutralizing pathogens like bacteria, viruses, parasites or fungi that have entered the body, and removing them from the body
• Recognizing and neutralizing harmful substances from the environment
• Fighting against the body’s own cells that have changed due to an illness, for example cancerous cells.

The normal adult spleen contributes to the homeostasis of the body by removing from the blood useless or potentially injurious materials (e.g., abnormal or “wornout” red blood cells and microorganisms) and by synthesizing immunoglobulins and properdin ⁸.

Figure 3. Spleen anatomy

Blood capillaries in the red pulp are quite permeable. Red blood cells can squeeze through the pores in these capillary walls and enter the venous sinuses. The older, more fragile red blood cells may rupture during this passage, and the resulting cellular debris is removed by phagocytic macrophages in the venous sinuses. These macrophages also engulf and destroy foreign particles, such as bacteria, that may be carried in the blood as it flows through the venous sinuses. Thus, the spleen filters blood much as the lymph nodes filter lymph.

Phagocytosis removes foreign particles from the lymph as it moves from the interstitial spaces to the bloodstream. Phagocytes in the blood vessels and in the tissues of the spleen (and the liver and bone marrow) remove particles that reach the blood. Monocytes that leave the bloodstream by diapedesis become macrophages. These large cells may be free, or fixed in various tissues. The fixed macrophages can divide and produce new macrophages. Neutrophils, monocytes, and macrophages constitute the mononuclear phagocytic system (reticuloendothelial system).

Fever is body temperature elevated above an individual’s normal temperature due to an elevated setpoint. It is part of the innate defense because as a result of the fever the body becomes inhospitable to certain pathogens. Higher body temperature causes the spleen (and the liver) to sequester iron, which reduces the level of iron in the blood. Because bacteria and fungi require iron for normal metabolism, their growth and reproduction in a fever-ridden body slows and may cease. Also, phagocytic cells attack more vigorously when the temperature rises. For these reasons, low-grade fever of short duration may be a natural response to infection, not a treated symptom.

T Cells and the Cellular Immune Response

A lymphocyte must be activated before it can respond to an antigen. T cell activation requires that processed fragments of the antigen be attached to the surface of another type of cell, called an antigen-presenting cell (accessory cell). Macrophages, B cells, and several other cell types can be antigen-presenting cells.

T cell activation may occur when a macrophage phagocytizes a bacterium and digests it within a phagolysosome formed by the fusion of the vesicle containing the bacterium (phagosome) and a lysosome. Some of the resulting bacterial antigens are then displayed on the macrophage’s cell membrane near certain protein molecules that are part of a group of proteins called the major histocompatibility complex (MHC). MHC antigens help T cells recognize that a newly displayed antigen is foreign (nonself).

Activated T cells interact directly with antigen-bearing cells. Such cell-to-cell contact is called the cellular immune response, or cell-mediated immunity. T cells (and some macrophages) also synthesize and secrete polypeptides called cytokines that enhance certain cellular responses to antigens. For example, interleukin-1 and interleukin-2 stimulate the synthesis of several other cytokines from other T cells. Additionally, interleukin-1 helps activate T cells, whereas interleukin-2 causes T cells to proliferate. This proliferation increases the number of T cells in a clone, which is a group of genetically identical cells that descend from a single, original cell. Other cytokines, called colony stimulating factors (CSFs), stimulate leukocyte production in red bone marrow and activate macrophages. T cells may also secrete toxins that kill their antigen-bearing target cells, growth-inhibiting factors that prevent target cell growth, or interferon that inhibits the proliferation of viruses and tumor cells. Several types of T cells have distinct functions.

A specialized type of T cell, called a helper T cell, is activated when its antigen receptor combines with a displayed foreign antigen. Once activated, the helper T cell proliferates and the resulting cells stimulate B cells to produce antibodies that are specific for the displayed antigen.

Another type of T cell is a cytotoxic T cell, which recognizes and combines with nonself antigens that cancerous cells or virally infected cells display on their surfaces near certain MHC proteins. Cytokines from helper T cells activate the cytotoxic T cell. Next, the cytotoxic T cell proliferates. Cytotoxic T cells then bind to the surfaces of antigen-bearing cells, where they release perforin protein that cuts pore like openings in the cell membrane, destroying these cells. In this way, cytotoxic T cells continually monitor the body’s cells, recognizing and eliminating tumor cells and cells infected with viruses. Cytotoxic T cells provide much of the body’s defense against HIV infection.

Some cytotoxic T cells do not respond to a nonself antigen on first exposure, but remain as memory T cells that provide for future immune protection. Upon subsequent exposure to the same antigen, these memory cells immediately divide to yield more cytotoxic T cells and helper T cells, often before symptoms arise.

Figure 4. T-cell (T lymphocyte) activation

Steps in Antibody Production

T Cell (T Lymphocyte) Activities

1. Antigen-bearing agents enter tissues.
2. An accessory cell, such as a macrophage, phagocytizes the antigen-bearing agent, and the macrophage’s lysosomes digest the agent.
3. Antigens from the digested antigen-bearing agents are displayed on the membrane of the accessory cell.
4. Helper T cell becomes activated when it encounters a displayed antigen that fits its antigen receptors.
5. Activated helper T cell releases cytokines when it encounters a B cell that has previously combined with an identical antigen-bearing agent.
6. Cytokines stimulate the B cell to proliferate, enlarging its clone.
7. Some of the newly formed B cells give rise to cells that differentiate into antibody-secreting plasma cells.

Figure 5. B (lymphocyte)-cell activation

B Cell (B Lymphocyte) Activities

1. Antigen-bearing agents enter tissues.
2. B cell encounters an antigen that fits its antigen receptors.
3. Either alone or more often in conjunction with helper T cells, the B cell is activated. The B cell proliferates, enlarging its clone.
4. Some of the newly formed B cells differentiate further to become plasma cells.
5. Plasma cells synthesize and secrete antibodies whose molecular structure is similar to the activated B cell’s antigen receptors.

Antibody Actions

In general, antibodies react to antigens in three ways. Antibodies directly attack antigens, activate complement, or stimulate localized changes (inflammation) that help prevent the spread of pathogens or cells bearing foreign antigens.

In a direct attack, antibodies combine with antigens, causing them to clump (agglutination) or to form insoluble substances (precipitation). Such actions make it easier for phagocytic cells to recognize and engulf the antigen-bearing agents and eliminate them. In other instances, antibodies cover the toxic portions of antigen molecules and neutralize their effects (neutralization). However, under normal conditions, direct antibody attack is not as important as complement activation in protecting against infection.

When certain IgG or IgM antibodies combine with antigens, they expose reactive sites on antibody molecules. This triggers a series of reactions, leading to activation of the complement proteins, which in turn produce a variety of effects. These include:

• coating the antigen-antibody complexes (opsonization), making the complexes more susceptible to phagocytosis;
• attracting macrophages and neutrophils into the region (chemotaxis);
• rupturing membranes of foreign cells (lysis); agglutination of antigen-bearing cells; and
• neutralization of viruses by altering their molecular structure, making them harmless.

Other proteins promote inflammation, which helps prevent the spread of infectious agents.

Immune Responses

Activation of B cells or T cells after they first encounter the antigens for which they are specialized to react constitutes a primary immune response. During such a response, plasma cells release antibodies (IgM, followed by IgG) into the lymph. The antibodies are transported to the blood and then throughout the body, where they help destroy antigen bearing agents. Production and release of antibodies continues for several weeks.

Following a primary immune response, some of the B cells produced during proliferation of the clone remain dormant as memory cells. If the same antigen is encountered again, the clones of these memory cells enlarge, and they can respond rapidly by producing IgG to the antigen to which they were previously sensitized. These memory B cells, along with the memory cytotoxic T cells, produce a secondary immune response.

As a result of a primary immune response, detectable concentrations of antibodies usually appear in the blood plasma five to ten days after exposure to antigens. If the same type of antigen is encountered later, a secondary immune response may produce the same antibodies within a day or two. Although newly formed antibodies may persist in the body for only a few months or years, memory cells live much longer.

Naturally acquired active immunity occurs when a person exposed to a pathogen develops a disease. Resistance to that pathogen is the result of a primary immune response.

A vaccine is a preparation that produces artificially acquired active immunity. A vaccine might consist of bacteria or viruses that have been killed or weakened so that they cannot cause a serious infection, or only molecules unique to the pathogens. A vaccine might also be a toxoid, which is a toxin from an infectious organism that has been chemically altered to destroy its dangerous effects. Whatever its composition, a vaccine includes the antigens that stimulate a primary immune response, but does not produce symptoms of disease and the associated infections.

Specific vaccines stimulate active immunity against a variety of diseases, including typhoid fever, cholera, whooping cough, diphtheria, tetanus, polio, chickenpox, measles (rubeola), German measles (rubella), mumps, influenza, hepatitis A, hepatitis B, and bacterial pneumonia. A vaccine has eliminated naturally acquired smallpox from the world.

Splenomegaly causes

A number of infections and diseases can cause an enlarged spleen. The enlargement might be temporary, depending on treatment:

• Cirrhosis and other diseases affecting the liver (hepatitis). Parenchymal liver disease causes increased vascular pressure leading to an increase in spleen size.
• Heart failure
• Portal or hepatic vein thrombosis. This leads to an increase in vascular pressure leading to splenomegaly.
• Malignancies and blood cancers (including lymphomas, acute and chronic leukemia, myeloproliferative disorders, metastatic solid tumors, and hemolytic anemias)
• Infectious including infection with Epstein Barr virus (infectious mononucleosis), cytomegalovirus, HIV, salmonella, Brucella, tuberculosis, malaria, toxoplasma and leishmania
• Bacterial infections, such as syphilis or an infection of your heart’s inner lining (bacterial endocarditis)
• Connective tissue diseases or autoimmune diseases. Chronic inflammatory conditions such as systemic lupus erythematosus (SLE), sarcoidosis and rheumatoid arthritis can cause an overactive immune response and spleen hyperplasia ², ³.
• Infiltrative disease including Gaucher’s disease, Amyloid, Niemann-Pick disease and Glycogen storage diseases.
• Splenic sequestration (pediatric sickle cell disease, hemolytic anemias, thalassemias)
• Miscellaneous including sarcoidosis, systemic lupus erythematous, autoimmune hemolytic anemia, immune-mediated neutropenia (immune-mediated destruction of red blood cells, white blood cells, or platelets), Felty’s syndrome, metastases, abscess, histiocytosis, trauma, hemangiomas, rare drug reactions (RhoGam) and cysts

The mechanism underlying splenic enlargement varies based on the cause. In the case of acute infectious illness, the spleen performs increased work in clearing antigens and producing antibodies and increases the number of reticuloendothelial cells contained within the spleen. These increased immune functions may result in splenic hyperplasia. In the case of liver disease and congestion, underlying illness causes increased venous pressure causing congestive splenomegaly. Extramedullary hematopoiesis exhibited in myeloproliferative disorders can lead to splenic enlargement (infiltrative splenomegaly) ¹⁰, ¹¹.

Splenic sequestration crisis is a life-threatening illness common in pediatric patients with homozygous sickle cell disease and beta thalassemia. Up to 30% of these children may develop splenic sequestration crisis with a mortality rate of up to 15%. This crisis occurs when splenic vaso-occlusion causes a large percentage of total blood volume to become trapped within the spleen. Clinical signs include severe, rapid drop in hemoglobin leading to hypovolemic shock and death. Pediatric patients with sickle cell disease and beta thalassemia experience multiple splenic infarcts, resulting in splenic fibrosis and scarring. Over time, this leads to a small, auto infarcted spleen typically by the time patients reach adulthood. Splenic sequestration crisis can only occur in functioning spleens which may be why this crisis is rarely seen in adults. However, late adolescent or adult patients in this group who maintain splenic function may develop splenic sequestration crisis.

Risk factors for enlarged spleen

Anyone can develop an enlarged spleen at any age, but certain groups are at higher risk, including:

• Children and young adults with infections, such as infectious mononucleosis caused by Epstein-Barr virus (EBV).
• People who have Gaucher’s disease, Niemann-Pick disease, and several other inherited metabolic disorders affecting the liver and spleen
• People who live in or travel to areas where malaria is common.

Splenomegaly symptoms

You may not be able to tell if you have an enlarged spleen. There are usually no symptoms from an enlarged spleen in some cases.

Symptoms of splenomegaly include:

• Upper left abdominal pain. It may also radiate to your left shoulder or back.
• Palpable spleen. You usually can’t feel your spleen with your hand, unless it’s enlarged.
• Loss of appetite or early fullness or inability to eat a large meal. Your enlarged spleen might encroach on your stomach below.
• Feeling full without eating or after eating only a small amount from the enlarged spleen pressing on your stomach
• Hiccups
• Pain or fullness in the left upper abdomen that may spread to the left shoulder.

If your spleen is beginning to malfunction, you may notice:

• Anemia. Symptoms of anemia, such as weakness and fatigue.
• More frequent colds or infections.
• Easy bleeding and bruising.

Commonly, patients will present with symptoms due to the underlying illness causing splenomegaly. Constitutional symptoms such as weakness, weight loss, and night sweats suggest malignant illness. Patients with splenomegaly due to acute infection may present with fever, rigors, generalized malaise, or focal infectious symptoms. Patients with underlying liver disease may present with symptoms related to alcohol abuse or hepatitis. Symptoms of anemia (lightheadedness, fatigue, weakness, pale skin, dizziness, headaches, shortness of breath, and cold hands and feet), easy bruising, bleeding, or petechiae may indicate splenomegaly due to underlying hemolytic process.

See your doctor promptly if you have pain in your left upper abdomen, especially if it’s severe or the pain gets worse when you take a deep breath.

Splenomegaly complications

Potential complications of an enlarged spleen are:

• Infection. An enlarged spleen can reduce the number of healthy red blood cells, platelets and white cells in your bloodstream, leading to more frequent infections. Anemia and increased bleeding also are possible.
• Ruptured spleen. Even healthy spleens are soft and easily damaged, especially in car crashes. The possibility of rupture is much greater when your spleen is enlarged. A ruptured spleen can cause life-threatening bleeding into your abdominal cavity.
• Tissue death. A severely enlarged spleen may outgrow its own blood supply. When blood can’t reach the tissues, they will stop functioning or die.
• Hypersplenism. An enlarged spleen may become overactive, trapping or removing too many blood cells from circulation. This can lead to anemia, low white blood cell count or low platelet count.

Splenomegaly diagnosis

An enlarged spleen usually doesn’t cause symptoms. An enlarged spleen is usually detected during a physical exam. A doctor usually can’t feel the spleen in an adult unless it’s enlarged. Your doctor can often feel it by gently examining your left upper abdomen. However, in some people — especially those who are slender — a healthy, normal-sized spleen can sometimes be felt during an exam.

Your doctor may confirm the diagnosis of an enlarged spleen with one or more of these tests:

• Blood tests, such as a complete blood count to check the number of red blood cells, white blood cells and platelets in your system. Blood tests can test for specific diseases, cancers, blood disorders and liver function problems.
• Ultrasound or computerized tomography (CT) scan to help determine the size of your spleen and whether it’s crowding other organs
• Magnetic resonance imagining (MRI) to trace blood flow through the spleen
• Bone marrow analysis. Your doctor might take a bone marrow aspiration and/or bone marrow biopsy to test the blood cell content in your bone marrow tissues. This can give your doctor information about how your spleen is functioning and can indicate certain disorders.

Imaging tests aren’t always needed to diagnose an enlarged spleen. But if your doctor recommends imaging, you generally don’t need any special preparation for an ultrasound or MRI.

If you’re having a CT scan, however, you may need to refrain from eating before the test. If you need to prepare, your doctor will let you know well in advance.

Finding the cause

Sometimes you may need more testing to find the cause of an enlarged spleen, including liver function tests and a bone marrow exam. These tests can provide more-detailed information about your blood cells than can blood drawn from a vein.

A sample of solid bone marrow is sometimes removed in a procedure called a bone marrow biopsy. Or you may have a bone marrow aspiration, which removes the liquid portion of your marrow. In many cases, both procedures are performed at the same time (bone marrow exam).

Both the liquid and solid bone marrow samples are usually taken from the pelvis. A needle is inserted into the bone through an incision. You’ll receive either general or local anesthesia before the test to ease discomfort.

A needle biopsy of the spleen is very rare because of the risk of bleeding.

Occasionally, your doctor may recommend surgery to remove your spleen when there’s no identifiable cause for the enlargement. After surgical removal, the spleen is examined under a microscope to check for possible lymphoma of the spleen.

Splenomegaly treatment

Treatment for an enlarged spleen focuses on the underlying problem or what’s causing your enlarged spleen and protecting you from complications of splenomegaly itself. For example, if you have a bacterial infection, treatment will include antibiotics.

Patients with splenomegaly from any cause are at increased risk of splenic rupture, and increased attention must be made to protect the patient from abdominal trauma. Treatment ranges from abdominal injury avoidance in the young healthy patient with splenomegaly due to infectious mononucleosis, to splenectomy of a massively enlarged spleen in a patient with Hairy cell leukemia. Likewise, the prognosis is largely dependent on underlying disease state ¹².

Patients who undergo splenectomy are at increased risk of overwhelming infection due to encapsulated organisms such as Haemophilus influenzae, Streptococcus pneumoniae, and Neisseria meningitidis. They should receive vaccinations against these organisms. Careful attention must be paid to post-splenectomy patients presenting with febrile illnesses as they may require more aggressive, empiric antibiotic therapy.

Watchful waiting

If you have an enlarged spleen but don’t have symptoms and the cause can’t be found, your doctor might suggest watchful waiting. You see your doctor for reevaluation in 6 to 12 months or sooner if you develop symptoms.

Spleen removal surgery

If an enlarged spleen causes serious complications or the cause can’t be identified or treated, surgery to remove your spleen (splenectomy) might be an option. In chronic or critical cases, surgery might offer the best hope for recovery.

Elective spleen removal requires careful consideration. You can live an active life without a spleen but you will have reduced immunity and you’re more likely to get serious or even life-threatening infections after spleen removal. Your doctor will recommend certain vaccines to protect you against some of the most common infections you may be more vulnerable to. Your doctor will recommend you wear a medical ID bracelet alerting medical professionals to your absent spleen. Your doctor may prescribe stronger antibiotics when you do get sick.

Reducing infection risk after surgery

After spleen removal, certain steps can help reduce your risk of infection, including:

• A series of vaccinations before and after the splenectomy. These include the pneumococcal (Pneumovax 23), meningococcal and haemophilus influenzae type b (Hib) vaccines, which protect against pneumonia, meningitis and infections of the blood, bones and joints. You’ll also need the pneumococcal vaccine every five years after surgery.
• Taking penicillin or other antibiotics after your surgery and anytime you or your doctor suspects the possibility of an infection.
• Calling your doctor at the first sign of a fever, which could indicate an infection.
• Avoiding travel to parts of the world where certain diseases, such as malaria, are common.

Lifestyle changes

If you have a chronically enlarged spleen, be careful to avoid trauma to your abdomen. An enlarged spleen is more vulnerable to rupture. It’s best to avoid high-contact sports. Your spleen may also be at risk of losing its functionality, or of becoming overactive. Look out for signs of anemia, such as paleness and fatigue. Your doctor may want to check your blood levels periodically.

In rare cases, an injury can rupture the spleen. If you have splenomegaly, your doctor may advise you to avoid contact sports. Your doctor will tell you what else you need to do to take care of yourself and any medical condition.

Avoid contact sports — such as soccer, football and hockey — and limit other activities as recommended by your doctor. Modifying your activities can reduce the risk of a ruptured spleen.

It’s also important to wear a seat belt. If you’re in a car accident, a seat belt can help prevent injury to your spleen.

Finally, be sure to keep your vaccinations up to date because your risk of infection is increased. That means at least an annual flu shot, and a tetanus, diphtheria and pertussis booster every 10 years. Ask your doctor if you need any additional vaccines.

Splenomegaly prognosis

The prognosis for patients with splenomegaly depends on the condition causing the enlargement. Regardless of the underlying cause, the risk of rupture, even with minor trauma, is high in patients with an enlarged spleen. If you have splenomegaly, your doctor may advise you to avoid contact sports. Your doctor will tell you what else you need to do to take care of yourself and any medical condition.

Avoid contact sports — such as soccer, football and hockey — and limit other activities as recommended by your doctor. Modifying your activities can reduce the risk of a ruptured spleen.

It’s also important to wear a seat belt. If you’re in a car accident, a seat belt can help prevent injury to your spleen.

1. Chapman J, Goyal A, Azevedo AM. Splenomegaly. [Updated 2023 Jun 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430907[↩][↩][↩]
2. Justiz Vaillant AA, Goyal A, Varacallo MA. Systemic Lupus Erythematosus. [Updated 2023 Aug 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK535405[↩][↩]
3. Chauhan K, Jandu JS, Brent LH, et al. Rheumatoid Arthritis. [Updated 2023 May 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441999[↩][↩]
4. Nguyen Y, Stirnemann J, Belmatoug N. La maladie de Gaucher : quand y penser ? [Gaucher disease: A review]. Rev Med Interne. 2019 May;40(5):313-322. French. doi: 10.1016/j.revmed.2018.11.012[↩][↩]
5. Kang DW, Kim SH. Clinical aspects of splenomegaly as a possible predictive factor of coronary artery changes in Kawasaki disease. Cardiol Young. 2019 Mar;29(3):297-302. doi: 10.1017/S1047951118002238[↩]
6. Gala AR, Surapaneni T, Aziz N, Kallur SD. A Review of Outcomes in Pregnant Women with Portal Hypertension. J Obstet Gynaecol India. 2018 Dec;68(6):447-451. doi: 10.1007/s13224-017-1016-1[↩]
7. Hijazi LS, Mead T. Functional Asplenism. [Updated 2018 Oct 27]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2018 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK499949[↩][↩]
8. Armitage JO. Spleen. In: Walker HK, Hall WD, Hurst JW, editors. Clinical Methods: The History, Physical, and Laboratory Examinations. 3rd edition. Boston: Butterworths; 1990. Chapter 150. Available from: https://www.ncbi.nlm.nih.gov/books/NBK258/[↩][↩][↩][↩]
9. Chapman J, Bhimji SS. Splenomegaly. [Updated 2017 May 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2017 Jun-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430907/[↩]
10. Palmiere C, Tettamanti C, Scarpelli MP, Tse R. The forensic spleen: Morphological, radiological, and toxicological investigations. Forensic Sci Int. 2018 Oct;291:94-99. doi: 10.1016/j.forsciint.2018.08.012. Epub 2018 Aug 18. Erratum in: Forensic Sci Int. 2019 Apr;297:384-387. doi: 10.1016/j.forsciint.2019.01.042[↩]
11. Sjoberg BP, Menias CO, Lubner MG, Mellnick VM, Pickhardt PJ. Splenomegaly: A Combined Clinical and Radiologic Approach to the Differential Diagnosis. Gastroenterol Clin North Am. 2018 Sep;47(3):643-666. doi: 10.1016/j.gtc.2018.04.009[↩]
12. Saab S, Brown RS. Management of Thrombocytopenia in Patients with Chronic Liver Disease. Dig. Dis. Sci. 2019 Apr 22[↩]