Overview

NCI Definition: A peripheral (mature) T-cell lymphoma, consisting of usually large anaplastic, CD30 positive cells. The majority of cases are positive for the anaplastic large cell lymphoma (ALK) protein. The most frequently seen genetic alteration is a t(2;5) translocation. Majority of patients present with advanced disease. The most important prognostic indicator is ALK positivity, which has been associated with a favorable prognosis. (WHO, 2001) [1]

Anaplastic large cell lymphomas most frequently harbor alterations in TET2, ZFHX3, XPO1, TP53, and TET1 [2].

Most Commonly Altered Genes in Anaplastic Large Cell Lymphoma

ZFHX3 Loss, XPO1 Q207E, XPO1 Mutation, TP53 c.217-c.1178 Missense, and TP53 c.142-c.212 Missense are the most common alterations in anaplastic large cell lymphoma [2].

Top Alterations in Anaplastic Large Cell Lymphoma

Significant Genes in Anaplastic Large Cell Lymphoma

ABL1 +

AFF1 +

ALK +

BCR +

CBFB +

CCND1 +

DEK +

ELL +

FLT3 +

IGH +

IKZF1 +

KIT +

KMT2A +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYH11 +

NPM1 +

NUP214 +

PBX1 +

RPN1 +

RUNX1 +

RUNX1T1 +

TCF3 +

TP53 +

Disease Details

Synonyms
CD30 Positive Anaplastic Large Cell Lymphoma, ALCL, Ki-1 Lymphoma, Ki-1+ ALCL, Ki-1+ Anaplastic Large Cell Lymphoma
Parent(s)
Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
Children
Primary Cutaneous Anaplastic Large Cell Lymphoma, Anaplastic Large Cell Lymphoma, ALK-Negative, and Anaplastic Large Cell Lymphoma, ALK-Positive
OncoTree Name
Anaplastic Large Cell Lymphoma
OncoTree Code
ALCL

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 4. 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.