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Lymphomatoid granulomatosis is a rare disorder characterized by an overproduction of white blood cells known as B lymphocytes. These B cells can build up in the tissues of the body, causing damage to the blood vessels. In many cases of lymphomatoid granulomatosis, the abnormal B cells contain the Epstein-Barr virus. The disease is more common in men, usually after the fifth decade of life. Lymphomatoid granulomatosis most commonly affects the lungs, though other areas of the body may also be affected. Signs and symptoms vary but can include cough, shortness of breath, tightness of the chest, fever, weight loss, and fatigue. Skin lesions and central nervous system changes such as headaches, seizures, and ataxia may also be seen. Rarely, the disorder can affect the kidneys or liver. The cause of the disorder is not well understood, though a combination of genetic and immune factors are thought to play a part. Treatment depends on the extent of the disease but may include interferon alfa-2b and combination chemotherapy with rituximab. Occasionally, the disorder resolves on its own without treatment. There has been some debate as to whether lymphomatoid granulomatosis should be viewed as a as a B-cell lymphoma or a lymphoproliferative disease or whether it should be viewed merely as a condition that can develop into a B-cell lymphoma. The prognosis is variable, though lymphomatoid granulomatosis can progress and become fatal in some cases.
Source: GARD Last updated on 05-01-20
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