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

/hy·per·splen·ism/ (-splen´izm) exaggeration of the hemolytic function of the spleen, resulting in deficiency of peripheral blood elements, hypercellularity of bone marrow, and splenomegaly.

hypersplenism

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

hypersplenism

[hī′pərsplē′nizəm]
Etymology: Gk, hyper + splen, spleen
a syndrome consisting of splenomegaly and a deficiency of one or more types of blood cells. Causes may include portal hypertension, the lymphomas, the hemolytic anemias, malaria, tuberculosis, and various connective tissue and inflammatory diseases. Patients complain of abdominal pain of the left upper and middle quadrant. Patients often experience a sensation of fullness after small meals secondary to an enlarged pressing against the stomach. On physical examination the enlarged spleen is felt and abnormal bruits (vascular sounds) may be auscultated over the epigastric area. Treatment of the underlying disorder may relieve the syndrome and its secondary effects. Splenectomy is considered when hemolytic anemias or splenic enlargement is severe, in treatment failures, or if the danger of vascular accident is significant. See also splenectomy.

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.

hypersplenism (hī´pərsplen´izəm),

n a syndrome consisting of splenomegaly and a deficiency of one or more types of blood cells.

hypersplenism

a condition characterized by splenomegaly and exaggeration of the phagocytic function of the spleen, resulting in deficiency of peripheral blood elements, and often by hypercellularity of the bone marrow.
References in periodicals archive ?
Hypersplenism or coexisting clinical conditions lead to their discovery.
The patient was seen in consultation for thrombocytopenia and hypersplenism in the hematology and hepatology clinics.
He had known splenomegaly with splenic infarctions by prior computed tomographic (CT) scanning and had mild chronic thrombocytopenia (platelets, 94,000-128,000/[micro]L), suggesting hypersplenism.
The most frequent sign is splenomegaly associated with hypersplenism leading to thrombocytopenia and anemia.
The relationship between altered MMP and TIMP expression in MNLs and chronic liver disease may be mediated by several factors, such as cytokine activation, viral load, or hypersplenism (30, 31).
Pancytopenia associated with brucellosis is attributed to hypersplenism, hemophagocytosis, and granulomatous lesions of the bone marrow, which is usually hypercellular (13), (15), (16).
His medical history was significant for chronic lymphocytic leukemia and a splenectomy performed secondary to hypersplenism and thrombocytopenia.
There are many causes of thrombocytopenia, including drugs, hypersplenism, and autoimmune disorders.
A significant increase in erythrocyte creatine was also observed in patients with liver cirrhosis, suggesting accelerated hemolysis because of hypersplenism and a possible abnormality of the erythrocyte membrane in cirrhotic patients (26).
In patients presenting with thrombocytopenia, diagnosis of ITP is made by exclusion of conditions that may cause thrombocytopenia, which include: drug use (generally heparin, alcohol, quinine/quinidine, sulphonamides), bacterial infections, viral infections (HIV, hepatitis, cytomegalovirus, Epstein-Barr virus), rickettsia infections, mycoplasma infections, lymphoproliferative diseases (chronic lymphocytic leukemia, large granular lymphocytic leukemia, lymphoma), autoimmune diseases (especially systemic lupus erythematosus, antiphospholipid antibody (APA) syndrome), disseminated intravascular coagulopathy, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, preeclampsia/eclampsia, HELLP syndrome, gestational thrombocytopenia, and hypersplenism.