Loading
5 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Lymphomatoid granulomatosis is a rare Epstein-Barr virus-driven disease of B cells that invades and destroys blood vessels, almost always in the lungs and often the brain and skin, in people whose immune control of the virus is weak. Low-grade disease can be treated with interferon and high-grade disease as a large B-cell lymphoma with rituximab-based chemotherapy.
WHO-HAEM5 lists lymphomatoid granulomatosis among the EBV-positive B-cell lymphoproliferative disorders, graded 1 to 3 by the number of large EBV-positive B cells, with grade 3 overlapping large B-cell lymphoma (Alaggio 2022; Katzenstein 2010). It affects mainly middle-aged adults, men almost twice as often as women, with multiple bilateral lung nodules and extrapulmonary involvement of skin and nervous system in more than a third; mortality has been high and treatment was not well established (Katzenstein 2010). In the National Cancer Institute series of 55 patients, all had lung involvement, 38 percent central nervous system involvement, none nodal or marrow disease, and all had past EBV exposure with a low median viral load; the lesions were angiocentric and rich in T cells with variable EBV-positive B cells (Song 2015).
How it differs from its parent: it is a spectrum from a virus-driven immunodeficiency disorder to a lymphoma, defined by grade; it spares nodes and marrow; and its low-grade forms are treated by restoring immune control (interferon alfa) rather than by chemotherapy.
| Setting | Approach | Guideline |
|---|---|---|
| Grade 1 and 2 | Interferon alfa, with withdrawal of iatrogenic immunosuppression. | not mapped |
| Grade 3 | Rituximab-based chemotherapy as for large B-cell lymphoma. | not mapped |