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Lymphomatoid Granulomatosis

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Elaine S. Jaffe, MD, Chief, Hematopathology Section, Lab of Pathology, National Cancer Institute, and Wyndham H. Wilson, M.D, Ph.D, Senior Investigator, Metabolism Branch, National Cancer Institute, for assistance in the preparation of this report.

Synonyms of Lymphomatoid Granulomatosis

  • benign lymph angiitis and granulomatosis
  • malignant lymph angiitis and granulomatosis
  • pulmonary angiitis
  • pulmonary Wegener's granulomatosis

Disorder Subdivisions

  • No subdivisions found.

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 tightness. Other areas of the body such as the skin, kidneys or central nervous system are also frequently 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.

Organizations related to Lymphomatoid Granulomatosis

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