Splenomegaly

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Splenomegaly
Splenomegalie bei CLL (labeled).jpg
CT scan in a patient with chronic lymphocytic leukemia, showing splenomegaly. Yellow arrows point at the spleen.
Specialty General surgery

Splenomegaly is an enlargement of the spleen. [1] The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload (such as in hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusae, is an important sign of portal hypertension. [2]

Contents

Definition

Maximum dimension of the spleen on abdominal ultrasonography. Maximum length of spleen on ultrasonography.jpg
Maximum dimension of the spleen on abdominal ultrasonography.

The standard system for classifying splenomegaly on radiography is: [3] [4]

Also, a cutoff of a craniocaudal height of 13 cm is also used to define splenomegaly. [5] In addition, individual intervals have been established:

90% confidence interval of maximum spleen length by abdominal ultrasonography by height of the person [6]
HeightSpleen length
WomenMen
155 – 159 cm6.4 – 12 cm
160 – 164 cm7.4 - 12.2 cm8.9 - 11.3 cm
165 – 169 cm7.5 – 11.9 cm8.5 – 12.5 cm
170 – 174 cm8.3 – 13.0 cm8.6 – 13.1 cm
175 – 179 cm8.1 – 12.3 cm8.6 – 13.4 cm
180 – 184 cm9.3 – 13.4 cm
185 – 189 cm9.3 – 13.6 cm
190 – 194 cm9.7 – 14.3 cm
195 – 199 cm10.2 – 14.4 cm
AgeCutoff [7]
3 months6.0 cm
6 months6.5 cm
12 months7.0 cm
2 years8.0 cm
4 years9.0
6 years9.5 cm
8 years10.0 cm
10 years11.0 cm
12 years11.5 cm
15 years
  • 12.0 cm
    for girls
  • 13.0 cm
    for boys

For children, the cutoffs for splenomegaly are given in this table, when measuring the greatest length of the spleen between its dome and its tip, in the coronal plane through its hilum while breathing quietly. [7]

At autopsy, splenomegaly can be defined as a spleen weight above the upper limit of the standard reference range of 230 g (8.1 oz). [8] [9]

Splenomegaly refers strictly to spleen enlargement, and is distinct from hypersplenism, which connotes overactive function by a spleen of any size. Splenomegaly and hypersplenism should not be confused. Each may be found separately, or they may coexist. Clinically, if a spleen is palpable (felt via external examination), it means it is enlarged as it has to undergo at least twofold enlargement to become palpable. However, the tip of the spleen may be palpable in a newborn baby up to three months of age. [10]

Signs and symptoms

Symptoms may include abdominal pain, chest pain, chest pain similar to pleuritic pain when stomach, bladder or bowels are full, back pain, early satiety due to splenic encroachment, or the symptoms of anemia due to accompanying cytopenia.

Signs of splenomegaly may include a palpable left upper quadrant abdominal mass or splenic rub. It can be detected on physical examination by using Castell's sign, Traube's space percussion or Nixon's sign, but an ultrasound can be used to confirm diagnosis. In patients where the likelihood of splenomegaly is high, the physical exam is not sufficiently sensitive to detect it; abdominal imaging is indicated in such patients. [11]

In cases of infectious mononucleosis splenomegaly is a common symptom and health care providers may consider using abdominal ultrasonography to get insight into a person's condition. [12] However, because spleen size varies greatly, ultrasonography is not a valid technique for assessing spleen enlargement and should not be used in typical circumstances or to make routine decisions about fitness for playing sports. [12]

Causes

The most common causes of splenomegaly in developed countries are infectious mononucleosis, splenic infiltration with cancer cells from a hematological malignancy and portal hypertension (most commonly secondary to liver disease, and sarcoidosis). Splenomegaly may also come from bacterial infections, such as syphilis or an infection of the heart's inner lining (endocarditis). [13] Splenomegaly also occurs in mammals parasitized by Cuterebra fontinella . [14]

The possible causes of moderate splenomegaly (spleen <1000 g) are many, and include:

Splenomegaly grouped on the basis of the pathogenic mechanism
Increased functionAbnormal blood flowInfiltration
Removal of defective RBCs

Immune hyperplasia

Response to infection (viral, bacterial, fungal, parasitic)

Disordered immunoregulation

Extramedullary hematopoiesis

Organ Failure

Vascular

Infections

Metabolic diseases

Benign and malignant "infiltrations"

Normal spleen (in green) Gray1217.png
Normal spleen (in green)

The causes of massive splenomegaly (spleen >1000 g) are

Pathophysiology

Splenomegaly can be classified based on its pathophysiologic mechanism:

[1]

Diagnosis

Abdominal CT is the most accurate. The spleen needs to be 2–3 times larger than normal to be palpable below the costal margin in physical examination.

Treatment

If the splenomegaly underlies hypersplenism, a splenectomy is indicated and will correct the hypersplenism. However, the underlying cause of the hypersplenism will most likely remain; consequently, a thorough diagnostic workup is still indicated, as, leukemia, lymphoma and other serious disorders can cause hypersplenism and splenomegaly. After splenectomy, however, patients have an increased risk for infectious diseases.

Patients undergoing splenectomy should be vaccinated against Haemophilus influenzae , Streptococcus pneumoniae , and Meningococcus . They should also receive annual influenza vaccinations. Long-term prophylactic antibiotics may be given in certain cases.

As an adaptation

An enlarged spleen may be an inherited, adaptive trait selected in populations that need extra oxygen carry capacity such as deep sea divers. [21] [22]

See also

Related Research Articles

<span class="mw-page-title-main">Spleen</span> Organ recycling old red blood cells and also housing lymphocytes

The spleen is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes from Ancient Greek σπλήν (splḗn).

<span class="mw-page-title-main">Infectious mononucleosis</span> Common viral infectious disease

Infectious mononucleosis, also known as glandular fever, is an infection usually caused by the Epstein–Barr virus (EBV). Most people are infected by the virus as children, when the disease produces few or no symptoms. In young adults, the disease often results in fever, sore throat, enlarged lymph nodes in the neck, and fatigue. Most people recover in two to four weeks; however, feeling tired may last for months. The liver or spleen may also become swollen, and in less than one percent of cases splenic rupture may occur.

<span class="mw-page-title-main">Lymphocytosis</span> Medical condition

Lymphocytosis is an increase in the number or proportion of lymphocytes in the blood. Absolute lymphocytosis is the condition where there is an increase in the lymphocyte count beyond the normal range while relative lymphocytosis refers to the condition where the proportion of lymphocytes relative to white blood cell count is above the normal range. In adults, absolute lymphocytosis is present when the lymphocyte count is greater than 5000 per microliter (5.0 x 109/L), in older children greater than 7000 per microliter and in infants greater than 9000 per microliter. Lymphocytes normally represent 20% to 40% of circulating white blood cells. When the percentage of lymphocytes exceeds 40%, it is recognized as relative lymphocytosis.

<span class="mw-page-title-main">Splenectomy</span> Surgical removal of the spleen

A splenectomy is the surgical procedure that partially or completely removes the spleen. The spleen is an important organ in regard to immunological function due to its ability to efficiently destroy encapsulated bacteria. Therefore, removal of the spleen runs the risk of overwhelming post-splenectomy infection, a medical emergency and rapidly fatal disease caused by the inability of the body's immune system to properly fight infection following splenectomy or asplenia.

<span class="mw-page-title-main">Hereditary spherocytosis</span> Medical condition

Hereditary spherocytosis (HS) is a congenital hemolytic disorder wherein a genetic mutation coding for a structural membrane protein phenotype causes the red blood cells to be sphere-shaped (spherocytosis), rather than the normal biconcave disk shape. This abnormal shape interferes with the cells' ability to flex during blood circulation, and also makes them more prone to rupture under osmotic stress, mechanical stress, or both. Cells with the dysfunctional proteins are degraded in the spleen, which leads to a shortage of erythrocytes and results in hemolytic anemia.

<span class="mw-page-title-main">Portal hypertension</span> Abnormally increased portal venous pressure

Portal hypertension is defined as increased portal venous pressure, with a hepatic venous pressure gradient greater than 5 mmHg. Normal portal pressure is 1–4 mmHg; clinically insignificant portal hypertension is present at portal pressures 5–9 mmHg; clinically significant portal hypertension is present at portal pressures greater than 10 mmHg. The portal vein and its branches supply most of the blood and nutrients from the intestine to the liver.

Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism.

Hepatosplenomegaly is the simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly). Hepatosplenomegaly can occur as the result of acute viral hepatitis, infectious mononucleosis, and histoplasmosis or it can be the sign of a serious and life-threatening lysosomal storage disease. Systemic venous hypertension can also increase the risk for developing hepatosplenomegaly, which may be seen in those patients with right-sided heart failure.

Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a type of myeloproliferative neoplasm, a group of cancers in which there is activation and growth of mutated cells in the bone marrow. This is most often associated with a somatic mutation in the JAK2, CALR, or MPL genes. In PMF, the bony aspects of bone marrow are remodeled in a process called osteosclerosis; in addition, fibroblast secrete collagen and reticulin proteins that are collectively referred to as (fibrosis). These two pathological processes compromise the normal function of bone marrow resulting in decreased production of blood cells such as erythrocytes, granulocytes and megakaryocytes, the latter cells responsible for the production of platelets.

Organomegaly is the abnormal enlargement of organs. For example, cardiomegaly is enlargement of the heart. Visceromegaly is the enlargement of abdominal organs. Examples of visceromegaly are enlarged liver (hepatomegaly), spleen (splenomegaly), stomach, kidneys, and pancreas.

<span class="mw-page-title-main">Splenic injury</span> Injury to the spleen

A splenic injury, which includes a ruptured spleen, is any injury to the spleen. The rupture of a normal spleen can be caused by trauma, such as a traffic collision.

<span class="mw-page-title-main">Castell's sign</span> Medical sign assessed to evaluate splenomegaly

Castell's sign is a medical sign assessed to evaluate splenomegaly and typically part of an abdominal examination. It is an alternative physical examination maneuver to percussion over Traube's space.

Felty's syndrome (FS), also called Felty syndrome, is a rare autoimmune disease characterized by the triad of rheumatoid arthritis, enlargement of the spleen and low neutrophil count. The condition is more common in those aged 50–70 years, specifically more prevalent in females than males, and more so in Caucasians than those of African descent. It is a deforming disease that causes many complications for the individual.

<span class="mw-page-title-main">Splenic infarction</span> Medical condition

Splenic infarction is a condition in which blood flow supply to the spleen is compromised, leading to partial or complete infarction in the organ. Splenic infarction occurs when the splenic artery or one of its branches are occluded, for example by a blood clot.

Splenic marginal zone lymphoma (SMZL) is a type of marginal zone lymphoma, a cancer made up of B-cells that replace the normal architecture of the white pulp of the spleen. The neoplastic cells are both small lymphocytes and larger, transformed lymphoblasts, and they invade the mantle zone of splenic follicles and erode the marginal zone, ultimately invading the red pulp of the spleen. Frequently, the bone marrow and splenic hilar lymph nodes are involved along with the peripheral blood. The neoplastic cells circulating in the peripheral blood are termed villous lymphocytes due to their characteristic appearance.

<span class="mw-page-title-main">Accessory spleen</span> Small nodule found apart from the main body of the spleen

An accessory spleen is a small nodule of splenic tissue found apart from the main body of the spleen. Accessory spleens are found in approximately 10 percent of the population and are typically around 1 centimetre in diameter. They may resemble a lymph node or a small spleen. They form either by the result of developmental anomalies or trauma. They are medically significant in that they may result in interpretation errors in diagnostic imaging or continued symptoms after therapeutic splenectomy. Polysplenia is the presence of multiple accessory spleens rather than one normal spleen.

<span class="mw-page-title-main">Wandering spleen</span> Medical condition

Wandering spleen is a rare medical disease caused by the loss or weakening of the ligaments that help to hold the spleen stationary.

<span class="mw-page-title-main">Abdominal ultrasonography</span>

Abdominal ultrasonography is a form of medical ultrasonography to visualise abdominal anatomical structures. It uses transmission and reflection of ultrasound waves to visualise internal organs through the abdominal wall. For this reason, the procedure is also called a transabdominal ultrasound, in contrast to endoscopic ultrasound, the latter combining ultrasound with endoscopy through visualize internal structures from within hollow organs.

<span class="mw-page-title-main">Littoral cell angioma</span> Medical condition

Littoral cell angioma, abbreviated LCA, and formally known as littoral cell angioma of the spleen, is a benign tumour of the spleen that arises from the cells that line the red pulp.

<span class="mw-page-title-main">Spleen pain</span> Human pain from the area of the spleen

Spleen pain is a pain felt from the left upper quadrant of the abdomen or epigastrium where the human spleen is located or neighboring.

References

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