Overview

NCI Definition: An acute myeloid leukemia (AML) in which abnormal promyelocytes predominate. It is characterized by the PML-RARA fusion. There are two variants: the typical and microgranular variant. This AML is particularly sensitive to treatment with all trans-retinoic acid and has a favorable prognosis. (WHO) [1]

Acute promyelocytic leukemias most frequently harbor alterations in FLT3, RUNX1, ASXL1, TET2, and NRAS [2].

Most Commonly Altered Genes in Acute Promyelocytic Leukemia

FLT3 Mutation, FLT3 Exon 14 Mutation, RUNX1 c.170-c.1439 Missense, RUNX1 Mutation, and NRAS Mutation are the most common alterations in acute promyelocytic leukemia [2].

Top Alterations in Acute Promyelocytic Leukemia

Significant Genes in Acute Promyelocytic Leukemia

ABL1 +

AFF1 +

BCR +

CBFB +

DEK +

ELL +

FLT3 +

IDH1 +

KIT +

KMT2A +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYH11 +

NPM1 +

NRIP3 +

NUP214 +

PML +

RARA +

RPN1 +

RUNX1 +

RUNX1T1 +

TP53 +

Disease Details

Synonyms
APML, APML - Acute promyelocytic leukemia, APL with PML-RARA, Acute Promyelocytic Leukemia with t(15;17)(q22;q12); PML-RARA, AML with t(15;17)(q22;q12), Acute Promyelocytic Leukemia with t(15;17)(q22;q12); PML/RARA, Promyelocytic Leukemia, FAB M3, APL
Parent(s)
Acute Myeloid Leukemia with Recurrent Genetic Abnormalities
OncoTree Name
APL with PML-RARA
OncoTree Code
APLPMLRARA

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.