Lymphomatoid Granulomatosis is also known as Angiocentric Lymphoma. It is a rare precancerous condition which is triggered by the Epstein- Barr Virus (EBV) infection.It is indicated by an overpopulation of white blood cells, mainly the B-lymphocytes. The abnormal cells then infiltrate the tissues and accumulate, causing lesions in the lungs, central nervous system, skin, liver and kidneys. Lymphomatoid Granulomatosis mainly affects the lungs and also affects other organs of the body. Although this condition is rare, it can be debilitating. It affects males twice as often as females and can occur at any age.
The exact cause of Lymphomatoid Granulomatosis is unknown. Meanwhile, Immune System Dysfunction, as seen in individuals with Human Immunodeficiency Virus (HIV) infection and Wiskott-Aldrich Syndrome, can increase the risk of Lymphomatoid Granulomatosis.
The symptoms are cough, fever, fatigue, shortness of breath, unintended weight loss, seizures, tightness of the heart, headaches, skin lesions and paralysis of one side of the body.
If left untreated, Lymphomatoid Granulomatosis can lead to aggressive and fatal B-cell lymphoma, organ infiltration, dysfunction of affected organs and hepatomegaly.
The laboratory diagnosis of Lymphomatoid Granulomatosis is through a biopsy of the lungs or other affected organs. It can be managed through chemotherapy and immunotherapy.
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References:
www.ncbi.nlm.nih.gov
www.rarediseases.org