Secondary Logo

Journal Logo

Institutional members access full text with Ovid®

Lymphomatoid Granulomatosis

Roschewski, Mark MD*; Wilson, Wyndham H. MD, PhD

doi: 10.1097/PPO.0b013e31826c5e19
Reviews
Buy

Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease. The atypical lymphoid cells directly accumulate within affected tissues and clinically present in the form of infiltrative lesions. It is usually a progressive disorder that virtually always involves the lung and characteristically presents as bilateral pulmonary nodules. Other commonly affected organ systems include the skin, central nervous system, and kidneys. The rareness of LYG in conjunction with its nonspecific presentation contributes to delays in diagnosis in many situations. Pathologically, it is characterized by the presence of an angiocentric and angiodestructive accumulation of varying numbers of T cells with varying numbers of atypical clonal EBV-positive B cells in a polymorphous inflammatory background. It can be subclassified using a grading system based on the number of EBV-positive large B-cell malignant cells, which is critical in selecting appropriate management strategies. Lower-grade LYG occasionally undergoes spontaneous remission and is best managed with strategies designed to enhance the host’s underlying immune system, whereas high-grade LYG is best managed by combination chemoimmunotherapy but has inferior outcomes. Lymphomatoid granulomatosis can lead to progressive pulmonary failure, central nervous system disease, or progression to overt EBV-positive lymphoma without appropriate recognition and management. Improvements in the modern understanding of the biology of LYG, particularly the precise role of EBV in its pathogenesis, offer promise in the development of improved management strategies.

From the *Hematologic Malignancy Section, Walter Reed National Military Medical Center, Bethesda, MD; and †Lymphoma Therapeutics Section, Metabolism Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.

Disclaimer: The views expressed in this paper are those of the authors and do not reflect the official policy of the Department of Army, Department of Defense, National Institutes of Health, or US government. We certify that all individuals who qualify as authors have been listed; each has participated in the conception and design of this work, the writing of the document, and the approval of the submission of this version; that the document represents valid work; that if we used information derived from another source, we obtained all necessary approvals to use it and made appropriate acknowledgments in the document; and that each takes public responsibility for it.

Conflicts of Interest and Sources of Funding: All research support comes from the intramural research program of the NIH.

The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Reprints: Wyndham H. Wilson, MD, PhD, Lymphoma Therapeutics Section, Metabolism Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Dr, Bethesda, MD 20892. E-mail: wilsonw@mail.nih.gov.

© 2012 Lippincott Williams & Wilkins, Inc.