What is Lymphomatoid Granulomatosis?

The rare neoplastic disease, lymphomatoid granulomatosis, typically develops in certain white blood cells contained within the blood and lymphatic systems. The abnormal cells proliferate beyond normal levels and circulate throughout the body, accumulating in the blood vessels and various body tissues. The disease produces a variety of symptoms depending on the location of abnormal cell aggregation. The cellular destruction caused by the lymphoproliferative disorder generally results in fatalities over the course of about five years, but some patients experience remission following treatment.

The disorder primarily affects the B cells of the immune system, which normally produce antibodies against organisms perceived as a threat. Researchers believe the Epstein-Barr virus may be responsible for lymphomatoid granulomatosis cancer, as tissue samples typically reveal the presence of these viral cells. Since the disease affects B cell production and development, scientists believe the affliction is similar to non-Hodgkin’s lymphoma. The abnormal cells may congregate anywhere in the body, producing symptoms specific to a particular organ or system but often deposited in the central nervous system, lungs and skin.

Patients usually experience fever and weight loss accompanied by a general feeling of illness. When the disorder infiltrates the lungs, they may have difficulty breathing and a chest-tightening sensation. Discolored phlegm and a productive cough are generally signs of a secondary infection or bleeding from cellular destruction, and death often occurs due to respiratory failure. Lymphomatoid granulomatosis might also produce lymphoid-related cutaneous conditions causing painful, raised, reddened areas on the skin. Cutaneous conditions might additionally include subsurface nodules, which eventually ulcerate.

If lymphomatoid granulomatosis infiltrates the central nervous system, the disease affects the blood vessels, brain and nerve tissue. Inflammation occurs and patients typically develop meningitis with symptoms that include behavioral, cognitive or sensory changes, seizures, paralysis or a lack of physical coordination. When the abnormal cells congregate in sufficient numbers in blood vessels, patients typically experience hypertension and as the pressure on vascular walls increases, the condition generally destroys blood vessels resulting in hemorrhage.

The disease usually develops more frequently in males, in persons over the age of 50 and in those having a weakened immune system. Health care providers generally diagnose lymphomatoid granulomatosis using computed tomography scans, magnetic resonance imaging and tissue biopsies. Depending on the stage of the disease process, physicians might use corticosteroids to reduce inflammation along with chemotherapy, radiation and surgery. Bone marrow transplants, antiviral medications or interferon therapy might also be implemented in the treatment of the disease.