title: [Lymphomatoid granulomatosis].
abstract: Lymphomatoid granulomatosis, described in 1972 by Liebow, is a rare, Epstein-Barr virus (EBV)-associated lymphoproliferative disorder, involving the lung, and often the skin or the central nervous system. It could have a systemic course making its diagnosis difficult. Controversy still remains about precise taxonomy and lymphomatoid granulomatosis is classified as a lymphoma, whose severity extends from indolent forms to aggressive large B cell lymphomas. Histology is essential and shows characteristically an inflammatory angiocentric infiltrate, composed with polymorphous mononucleated cells containing a varying number of large atypical CD20-positive B-lymphocytes within a background of numerous small reactive CD3-positive T-lymphocytes, often associated with necrosis. In situ hybridization often shows EBV RNA within atypical B-cells. Atypical large B-lymphocytes proportion and to a lesser degree EBV-positive B-lymphocytes proportion allow to classify the disease (grade I to III) and have a prognostic value. An aggressive form of B lymphoma occurs in 7 to 47% of cases during lymphomatoid granulomatosis course. Moreover, grade III diseases share numerous characteristics of lymphoma and often require chemotherapy. Several conditions mimic lymphomatoid granulomatosis, and include various hematologic malignancies (large B-cells lymphomas, T/NK lymphomas, post-immunodepression lymphoproliferative disorders) or granulomatosis with polyangiitis. The objective of this article is to review the clinical, radiological, histological and therapeutic characteristics of this rare disorder.
authors.affiliation: Service de médecine interne, centre hospitalier Robert-Bisson, 4, rue Roger-Aini, 14107 Lisieux, France. hubertdeboysson@yahoo.fr
authors.forename: H
authors.initials: H
authors.lastname: de Boysson
authors.forename: L
authors.initials: L
authors.lastname: Geffray
mesh_terms: D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D008223:Lymphoma / Q000145:classification / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D008230:Lymphomatoid Granulomatosis / Q000145:classification / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy