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What Is a Lymphoproliferative Disorder?

Lymphoproliferative disorders are typically indicated by a presence of extra white blood cells, which notes a malfunction in the immune system.
An MRI is one way to detect lymphoproliferative disorders.
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  • Written By: Dulce Corazon
  • Edited By: W. Everett
  • Last Modified Date: 21 October 2014
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A lymphoproliferative disorder is a medical condition characterized by the dysfunction of the immune system often resulting in excessive production of lymphocytes or white blood cells. The immune system is the body's defense against infections and diseases caused by invading organisms such as bacteria and viruses. People with these disorders often have compromised immune systems, and they are prone to develop severe infections. Malignancy or cancer may also develop as a result.

Lymphocytes are produced in the bone marrow and are usually found in the blood, lymph nodes, and spleen. Lymph nodes are distributed throughout the body to fight against infection. The spleen, located in the left upper section of the abdomen, stores blood and also protects the body against infection. In those with a lymphoproliferative disorder, increased lymphocytes in the bloodstream can lead to the enlargement of the lymph nodes and the spleen.

Many of these disorders originate from inherited conditions and sometimes are caused by acquired immune system dysfunction. Other cases, however, have no known cause. Examples include ataxia telangiectasia, Wiskott-Aldrich syndrome, and autoimmune lymphoproliferative syndrome (ALPS). These conditions are often a result of genetic mutations inherited from one or both parents, and may occur in both men and women.

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An X-linked lymphoproliferative syndrome is another inherited disorder that mostly affects men. Many men with this condition have increased susceptibility to develop infections due to the Epstein-Barr virus (EVB). EBV, also known as herpesvirus 4, is often the cause of infectious mononucleosis. Symptoms include lymph node enlargement, fever, and sore throat. These patients may develop lymphoma, a cancer affecting the immune system, and aplastic anemia, a condition marked by failure of the bone marrow to produce new blood cells.

One acquired cause of lymphoproliferative disorder in children includes an infection with the human immunodeficiency virus (HIV). HIV-infected mothers can pass the virus to their children during pregnancy, during delivery, or through breast milk. Disorders can also develop after organ transplant procedures and use of immunosuppressive drugs. Immunosuppressive drugs are administered in order to suppress the immune system from rejecting the new organ.

Diagnostic tools usually used to evaluate patients with lymphoproliferative disorders include a complete blood count (CBC), blood test to detect EBV infection, bone scan, X-ray, and magnetic resonance imaging (MRI). A team of professionals will often manage the patient, and can include oncologists, surgeons, and other medical specialists whose expertise may be needed, depending on the extent of the patient's condition.

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Discuss this Article

bear78
Post 3

A relative of mine was also suspected of having this disorder. But it was found that she had something called t-zone lymphoma. Apparently the two have similar symptoms and findings and doctors don't think of t-zone lymphoma right away because it is very rare.

SteamLouis
Post 2

My nephew has lymphoproliferative disorder. It was diagnosed when he was three years old.

When it develops due to a viral infections and diseases the article mentioned, it can happen at any age of course.

But for people like my nephew who have this genetic disorder from birth, it's diagnosed at a very early age. I think by the time the child is four or five years, it is diagnosed.

burcinc
Post 1

I can imagine how difficult it would be for the doctor and patient to treat this disorder when they are also trying to prevent the rejection of a transplanted organ.

I think the same is true for cancer patients who have to undergo chemotherapy and radiotherapy and also have lymphoproliferative disorder.

Both of these situations need urgent treatment and care. I wonder how doctors make decisions in these situations and which treatment will be given priority to and for how long.

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