Granulomatosis, Lymphomatoid

Synonyms

• Benign Lymph Angiitis and Granulomatosis
• Malignant Lymph Angiitis and Granulomatosis
• Pulmonary Angiitis
• Pulmonary Wegener's Granulomatosis

Disorder Subdivisions

• None

General Discussion

Lymphomatoid granulomatosis is a rare disorder characterized by overproduction (proliferation) of white blood cells called lymphocytes (lymphoproliferative disorder). The abnormal cells infiltrate and accumulate (form lesions or nodules) within tissues. The lesions or nodules damage or destroy the blood vessels within these tissues. The lungs are most commonly affected in lymphomatoid granulomatosis. Symptoms often include cough, shortness of breath (dyspnea), and chest pain. Other areas of the body such as the skin, kidneys or central nervous system may also be affected.



The abnormal cells in lymphomatoid granulomatosis are B-cells (B lymphocytes) containing the Epstein-Barr virus. There are two main types of lymphocytes: B-lymphocytes, which may produce specific antibodies to "neutralize" certain invading microorganisms, and T-lymphocytes, which may directly destroy microorganisms or assist in the activities of other lymphocytes. Because lymphomatoid granulomatosis is caused by the overproduction of abnormal B-cells, affected individuals may eventually develop B-cell lymphoma, a form of non-Hodgkin lymphoma. Lymphoma is a general term for cancer of the lymphatic system.

Resources

American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Avenue

Eastpointe, MI 48021-2227

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



American Cancer Society, Inc.

1599 Clifton Road NE

Atlanta, GA 30329

USA

Tel: (404)320-3333

Tel: (800)227-2345

Internet: http://www.cancer.org



American Lung Association

61 Broadway, 6th Floor

New York, NY 10006

USA

Tel: (212)315-8700

Fax: (212)315-8870

Tel: (800)586-4872

Internet: http://www.lungusa.org



Rare Cancer Alliance

1649 North Pacana Way

Green Valley, AZ 85614

USA

Tel: (520)625-5495

Fax: (615)526-4921

Email: sharon.lane@rare-cancer.org

Internet: http://www.rare-cancer.org



AutoImmunity Community

Tel: (919) 552-9057

Email: bandrews@autoimmunitycommunity.org

Internet: http://autoimmunitycommunity.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  3/4/2009

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