Overview

NCI Definition: An angiocentric and angiodestructive lymphoproliferative disease involving extranodal sites, comprised of Epstein-Barr virus (EBV)-positive B-cells admixed with reactive T-cells. Incidence is higher among adult males; patients with a history of immunodeficiency are at increased risk. The most common site of involvement is the lung; other common sites include brain, kidney, liver, and skin. Morphologically, three grades are recognized: grade I, II, and III. Grade III lymphomatoid granulomatosis should be approached clinically as a subtype of diffuse large B-cell lymphoma. [1]

Significant Genes in Lymphomatoid Granulomatosis

AFF1 +

ALK +

BCL2 +

BCL6 +

ELL +

IRF4 +

KMT2A +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

Disease Details

Synonyms
lymphomatoid granulomatosis, Lymphomatoid granulomatosis, LYG
Parent(s)
B-Cell Proliferation of Uncertain Malignant Potential
OncoTree Name
Lymphomatoid Granulomatosis
OncoTree Code
LYG

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.