Overview

NCI Definition: A rare extranodal B-cell non-Hodgkin lymphoma, characterized by the presence of lymphoma cells exclusively in the lumina of small vessels, particularly capillaries. This is an extremely aggressive lymphoma which responds poorly to chemotherapy. (WHO, 2001) -- 2003 [1]

Significant Genes in Intravascular Large B-Cell Lymphoma

AFF1 +

ALK +

BCL2 +

BCL6 +

ELL +

IRF4 +

KMT2A +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYC +

Disease Details

Synonyms
Malignant Angioendotheliomatosis, Angiotropic Large Cell Lymphoma, Intravascular B-Cell Lymphoma, Angiotropic Lymphoma
Parent(s)
Diffuse Large B-Cell Lymphoma
OncoTree Name
Intravascular Large B-Cell Lymphoma
OncoTree Code
IVBCL

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.