Related Experiment Videos
Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications
1Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
Cancer Surveys
|January 1, 1997
Summary
Lymphomatoid granulomatosis (LG) is an Epstein-Barr virus (EBV)-positive B-cell proliferation with T-cell involvement, often affecting extranodal sites like the lungs. While T-cells infiltrate, B-cells show clonal EBV, suggesting an EBV-driven disorder.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Lymphomatoid granulomatosis (LG) shares clinical and pathological similarities with angiocentric T/NK cell lymphoma.
- Recent findings classify LG as an Epstein-Barr virus (EBV)-positive B-cell proliferation accompanied by a significant T-cell response.
Purpose of the Study:
- To elucidate the underlying pathogenesis of Lymphomatoid granulomatosis.
- To differentiate LG from T/NK cell lymphomas based on cellular origin and viral association.
Main Methods:
- Analysis of cellular infiltrates and their gene rearrangements (T-cell receptor, VDJ PCR).
- Detection and localization of Epstein-Barr virus (EBV) sequences within B-cells.
- Clinical evaluation of immune defects, including cytotoxic T-cell function and CD8+ T-cell levels.
Main Results:
- LG involves extranodal sites, predominantly the lung, with characteristic necrosis.
- While T-cells are the main infiltrate, B-cells harbor clonal EBV sequences, indicating a B-cell origin.
- Patients often exhibit T-cell dysfunction and are susceptible in immunodeficiency states.
Conclusions:
- Lymphomatoid granulomatosis is an EBV-driven B-cell lymphoproliferative disorder, distinct from T/NK cell lymphomas.
- Understanding the pathogenesis, including the role of EBV and chemokines, is crucial for effective treatment.
- Therapeutic strategies may involve chemotherapy, interferon, and addressing underlying immunodeficiencies.