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. Subsets of cases of BL are also positive for EBV.
Lymphomatoid granulomatosis is a rare EBV-driven lymphoproliferative disorder involving extranodal sites in patients with inherited or acquired immunodeficiencies.
: a clinicopathologic study of 42 patients.
(LYG) is an angiocentric and angiodestructive process that commonly affects the lung as bilateral nodular infiltrates, mimicking Wegener granulomatosis both clinically and radiographically.
These include bronchial asthma, allergic broncho-pulmonary aspergillosis (ABPA), acute eosinophilic pneumonia, chronic eosinophilic pneumonia, idiopathic hypereosinophilic syndrome, Wegener's granulomatosis, lymphomatoid granulomatosis
, Churg-Strauss syndrome, some malignancies, and drug hypersensitivity reactions.
(16,17) It affects patients older than 50 years who do not have a known immunodeficiency and do not meet criteria for other EBV-associated processes or lymphomas (such as infectious mononucleosis, lymphomatoid granulomatosis
, and plasmablastic lymphoma).
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.
Angiocentric destruction observed in some forms of EBV-related, T cell-rich B-cell lymphoma (lymphomatoid granulomatosis
) is not a common feature in pyothorax-associated lymphoma.
(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
. (8) Some cases of sclerosing mediastinitis, which may secondarily affect the lung, have also been reported as a manifestation of IgG4 systemic sclerosing disease.
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.
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).