Overview

NCI Definition: An aggressive T-cell leukemia, characterized by the proliferation of small to medium sized prolymphocytes with a mature T-cell phenotype, involving the blood, bone marrow, lymph nodes, liver, spleen, and skin. (WHO, 2001) [1]

T-cell prolymphocytic leukemias most frequently harbor alterations in JAK3, ATM, TP53, STAT5B, and STAG2 [2].

Most Commonly Altered Genes in T-Cell Prolymphocytic Leukemia

JAK3 Mutation, ATM Mutation, TP53 c.217-c.1178 Missense, TP53 Mutation, and TP53 Missense are the most common alterations in T-cell prolymphocytic leukemia [2].

Top Alterations in T-Cell Prolymphocytic Leukemia

Significant Genes in T-Cell Prolymphocytic Leukemia

ABL1 +

AFF1 +

BCR +

DEK +

ELL +

FGFR3 +

FLT3 +

IGH +

KMT2A +

MAF +

MECOM +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

NRIP3 +

NUP214 +

PBX1 +

RPN1 +

TCF3 +

TP53 +

Disease Details

Synonyms
T Prolymphocytic Leukemia, T Cell Prolymphocytic Leukemia, T-prolymphocytic leukemia, T-PLL
Parent(s)
Prolymphocytic Leukemia
OncoTree Name
T-Cell Prolymphocytic Leukemia
OncoTree Code
TPLL

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 8. 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.