hypersplenism


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Hypersplenism

 

Definition

Hypersplenism is a type of disorder which causes the spleen to rapidly and prematurely destroy blood cells.

Description

The spleen is located in the upper left area of the abdomen. One of this organ's major functions is to remove blood cells from the body's bloodstream. In hypersplenism, its normal function accelerates, and it begins to automatically remove cells that may still be normal in function. Sometimes, the spleen will temporarily hold onto up to 90% of the body's platelets and 45% of the red blood cells. Hypersplenism may occur as a primary disease, leading to other complications, or as a secondary disease, resulting from an underlying disease or disorder. Hypersplenism is sometimes referred to as enlarged spleen (splenomegaly). An enlarged spleen is one of the symptoms of hypersplenism. What differentiates hypersplenism is its premature destruction of blood cells.

Causes and symptoms

Hypersplenism may be caused by a variety of disorders. Sometimes, it is brought on by a problem within the spleen itself and is referred to as primary hypersplenism. Secondary hypersplenism results from another disease such as chronic malaria, rheumatoid arthritis, tuberculosis, or polycythemia vera, a blood disorder. Spleen disorders in general are almost always secondary in nature. Hypersplenism may also be caused by tumors.
Symptoms of hypersplenism include easy bruising, easy contracting of bacterial diseases, fever, weakness, heart palpitations, and ulcerations of the mouth, legs and feet. Individuals may also bleed unexpectedly and heavily from the nose or other mucous membranes, and from the gastrointestinal or urinary tracts. Most patients will develop an enlarged spleen, anemia, leukopenia, or abnormally low white blood cell counts, or thrombocytopenia, a deficiency of circulating platelets in the blood. Other symptoms may be present that reflect the underlying disease that has caused hypersplenism.
An enlarged spleen can be caused by a variety of diseases, including hemolytic anemia, liver cirrhosis, leukemia, malignant lymphoma and other infections and inflammatory diseases. Splenomegaly occurs in about 10% of systemic lupus erythematosus patients. Sometimes, it is caused by recent viral infection, such as mononucleosis. An enlarged spleen may cause pain in the upper left side of the abdomen and a premature feeling of fullness at meals.

Diagnosis

Diagnosis of hypersplenism begins with review of symptoms and patient history, and careful feeling (palpation) of the spleen. Sometimes, a physician can feel an enlarged spleen. X-ray studies, such as ultrasound and computed tomography scan (CT scan), may help diagnose an enlarged spleen and possible underlying causes, such as tumors. Blood tests indicate decreases in white blood cells, red blood cells, or platelets. Another test measures red blood cells in the liver and spleen after injection of a radioactive substance, and indicates areas where the spleen is holding on to large numbers of red cells or is destroying them.
Enlarged spleens are diagnosed using a combination of patient history, physical examination, including palpation of the spleen, if possible, and diagnostic tests. A history of fever and systemic symptoms may be present because of infection, malaria, or an inflammatory disorder. A complete blood count is taken to check counts of young red blood cells. Liver function tests, CT scans, and ultrasound exams can also help to detect an enlarged spleen.

Treatment

In secondary hypersplenism, the underlying disease must be treated to prevent further sequestration or destruction of blood cells, and possible spleen enlargement. Those therapies will be tried prior to removal of the spleen (splenectomy), which is avoided if possible. In severe cases, the spleen must be removed. Splenectomy will correct the effects of low blood cell concentrations in the blood.

Key terms

Cirrhosis — Hardening of an organ, usually the liver. Cirrhosis of the liver is a progressive disease which leads to destruction of liver cells, interference with blood flow in the liver, and interference with the function of the liver.
Palpitations — Throbbing or pulsation. Heart palpitations usually infer an irregular or rapid rhythm.
Polycythemia vera — A chronic disorder characterized by increased red blood cell mass and other malfunctions of the blood system. It most commonly occurs in males of Jewish ancestry between the ages of 40 and 60.
Systemic — Relating to a system, or especially the entire system.
Systemic lupus erythematosus — A connective tissue disease that results in fever, weakness, fatigue, joint pain and arthritis.
Ulcerations — Breaks in skin or mucous membranes that are often accompanied by loss of tissue on the surface.

Prognosis

Prognosis depends on the underlying cause and progression of the disease. Left untreated, spleen enlargement can lead to serious complications. Hypersplenism can also lead to complications due to decreased blood cell counts.

Prevention

Some of the underlying causes of hypersplenism or enlarged spleen can be prevented, such as certain forms of anemia and cirrhosis of the liver due to alcohol. In other cases, the hypersplenism may not be preventable, as it is a complication to an underlying disorder.

Resources

Organizations

American Liver Foundation. 1425 Pompton Ave., Cedar Grove, NJ 07009. (800) 223-0179. http://www.liverfoundation.org.
American Society of Hematology. 1200 19th Street NW, Suite 300, Washington, DC 20036-2422. (202) 857-1118. http://www.hematology.org.
National Heart, Lung and Blood Institute. PO Box 30105, Bethesda, MD 20824-0105. (301) 251-1222. http://www.nhlbi.nih.gov.

hypersplenism

 [hi″per-splen´izm]
a condition characterized by exaggeration of the hemolytic function of the spleen, resulting in deficiency of peripheral blood elements, and by hypercellularity of the bone marrow and splenomegaly.

hy·per·splen·ism

(hī'pĕr-splēn'izm),
Any of a group of conditions in which the cellular components of the blood or platelets are removed at an abnormally high rate by the spleen, resulting in low circulating levels.

hypersplenism

(hī′pər-splĕn′ĭz′əm, -splē′nĭz′əm)
n.
A condition in which the hemolytic action of the spleen is greatly increased.

hypersplenism

Pathologic enlargement of the spleen, often accompanied by pancytopenia, hyperplasia of BM precursors, and improvement upon splenectomy Etiology hypersplenism Blood stasis often 2º to cirrhosis, thrombosis and other vascular abnormalities, anemia, lymphoproliferative disorders–eg, lymphoma, leukemia–especially CML, infection–eg, infectious mononucleosis, kala azar, malaria, TB; infiltrations–eg, Gaucher disease, Niemann-Pick disease, connective tissue disease Clinical If isolated, asymptomatic; primary diseases may be accompanied by malaise, abdominal pain, fever, fullness, purpura, hematemesis, GI bleeding Lab ↓ RBC survival, reticulocytosis, 'left shift' of myeloid series. Cf Splenosis.

hy·per·splen·ism

(hī'pĕr-splēn'izm)
Any condition in which the cellular components of the blood or platelets are removed at an abnormally high rate by the spleen.

hypersplenism

A condition in which an enlarged spleen is associated with a reduction in the levels of white cells and platelets in the blood. It is thought that the deficiency of these elements in the blood is due to their concentrating in the spleen.

hy·per·splen·ism

(hī'pĕr-splēn'izm)
Any of a group of conditions in which cellular components of blood or platelets are removed at an abnormally high rate by the spleen.
References in periodicals archive ?
The next common cause in this study was found to be dengue fever (21.7%) which may be due again to hypersplenism, hemophagocytosis, or immune hemolysis.
Mullen, "Prevalence of peripheral blood cytopenias (hypersplenism) in patients with nonalcoholic chronic liver disease," American Journal of Gastroenterology, vol.
Brown, "Management of hypersplenism by partial splenic embolization with ethylene vinyl alcohol copolymer," AJR American Journal of Roentgenology, vol.
Changes in the composition of hepatic bile include reduced bile acid synthesis and transport [75], diminished cholesterol secretion, increased hydrolysis of conjugated bilirubin in the bile, and chronic hemolysis due to hypersplenism, which induces increased secretion of unconjugated bilirubin [8, 20, 70]; all of these may induce pigment lithogenesis in CLD.
Spleen is also commonly involved in different hemoglobinopathies and usually manifest with splenomegaly, hypersplenism, acute splenic sequestration, and splenic infarction [7, 8].
A total of 49 cases were classified into three groups according to splenic function: a splenectomy group, a normal splenic function group (control group), and a hypersplenism group.
Overall, 14(56%) patients had hypersplenism. Endoscopic band ligations (EBL) were done in oesophageal variceal patients and were successfully managed for varices, while 5(20%) patients required portosystemic shunt surgeries.
Laparoscopic splenectomy in colorectal cancer patients with chemotherapyassociated thrombocytopenia due to hypersplenism. Case Rep Oncol.
Hypersplenism, abdominal discomfort due to massive splenomegaly and splenic injury are other indications of splenectomy in thalassemia patients.5 Splenectomy has its own complications such as post splenectomy sepsis, which is manageable with pre-operative vaccination and post-operative use of long acting antibiotics.1 This study was carried out with an objective to assess the outcome of splenectomy as a whole as regard to blood transfusion, abdominal discomfort, interval of transfusion, hospital visits and post splenectomy infection.
For cirrhotic patients, preoperative platelet count was generally lower than normal due to concurrent hypersplenism. Under such condition, removal of spleen would lead to a platelet rebound phenomenon and the soaring count and augmented aggregation competence of platelet after operation would contribute to the hypercoagulable state, which, in combination with other underlying causes, may synergistically result in the formation of PVST.
The cause of anemia is probably multifactorial: immune-mediated mechanism, alteration of red blood cells membrane permeability, hemolysis, and hypersplenism. The former is possibly the main explanation for low leukocyte and platelet counts.
In "human immunodeficiency virus infection", immune mechanisms in which anti-neutrophil antibodies and hypersplenism are involved are also effective.