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Several other large B-cell lymphomas are occasionally or always positive for EBV, including PBL, primary effusion lymphoma, DLBCL associated with chronic inflammation, and lymphomatoid granulomatosis.
Lymphomatoid granulomatosis is a rare EBV-driven lymphoproliferative disorder involving extranodal sites in patients with inherited or acquired immunodeficiencies.
Fatal haemoptysis in a case of lymphomatoid granulomatosis treated with rituximab.
Successful treatment of mediastinal lymphomatoid granulomatosis with rituximab monotherapy.
Establishing the diagnosis of lymphomatoid granulomatosis usually requires an open lung or video-assisted thoracoscopic biopsy; because of the focal nature of lymphomatoid granulomatosis and the fact that it is not bronchocentric, a low diagnostic yield with bronchoscopic or transbronchial biopsies is likely.
Lymphomatoid granulomatosis (LyG) is an EBV-driven lymphoproliferative disorder that typically affects adults with a known immunodeficiency, such as human immunodeficiency virus (HIV), iatrogenic immunosuppression, and Wiskott-Aldrich syndrome.
The differential diagnoses include lymphomatoid granulomatosis, primary CNS lymphoma, CD5+ diffuse large B-cell lymphoma, reactive lymphoid hyperplasia, CNS vasculitis, HPC-associated disorders other than IVLBCL, the acute leukemias, and lymphomas with an intravascular component.
Lymphomatoid granulomatosis is recognized as an angiocentric and angiodestructive disease arising in a variety of extranodal sites.
5-8) Reported pulmonary manifestations of IgG4-related sclerosing disease have included fibroinflammatory masses, the plasma cell-rich variant of inflammatory pseudotumor, an interstitial pneumonitis resembling idiopathic nonspecific interstitial pneumonia (NSIP), (7) and some lesions formerly thought to represent grade I lymphomatoid granulomatosis.
Past awards include the Arthur Purdy Stout Society for Surgical Pathologists (1995), the John Brinton Hill Award (1995), and the Charles Carrington Memorial Prize (1996) received for his work in lymphomatoid granulomatosis, the spectrum of p53 mutations in lung cancer, and the role of apoptosis in acute lung injury.
Lymphomatoid granulomatosis is included in this review because it is an EBV driven LPD that shares some morphologic and clinical features with EBV associated DLBCL and may progress to DLBCL.
For example, germinal centers are frequently encountered in extranodal lymphomas of the MALT type, and a mixture of cell types is characteristic of lymphomatoid granulomatosis (angiocentric immunoproliferative disorder).