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  • A neoplastic lymphoproliferative process characterized by an angiocentric arrangement of tumor cells and associated angiodestruction. It includes lymphomatoid granulomatosis which is a lymphoproliferative lesion derived from mature B-lymphocytes and cases of extranodal NK/T-cell lymphomas of nasal type.
  • An angiocentric and angiodestructive lymphoproliferative disease involving extranodal sites, comprised of Epstein-Barr virus (EBV)-positive B-cells admixed with reactive T-cells. Incidence is higher among adult males; patients with a history of immunodeficiency are at increased risk. The most common site of involvement is the lung; other common sites include brain, kidney, liver, and skin. Morphologically, three grades are recognized: grade I, II, and III. Grade III lymphomatoid granulomatosis should be approached clinically as a subtype of diffuse large B-cell lymphoma.
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