Overview

NCI Definition: A myelodysplastic/myeloproliferative neoplasm characterized by the principal involvement of the neutrophil series with leukocytosis and multilineage dysplasia. The neoplastic cells do not have a Philadelphia chromosome or the BCR/ABL fusion gene. (WHO, 2001) [1]

Atypical chronic myeloid leukemia, BCR-ABL1 negatives most frequently harbor alterations in SRSF2, KIT, U2AF1, SETBP1, and IDH1 [2].

Most Commonly Altered Genes in Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative

SRSF2 Mutation, SRSF2 Exon 1 Mutation, KIT Mutation, KIT Exon 17 Mutation, and KIT D816V are the most common alterations in atypical chronic myeloid leukemia, BCR-ABL1 negative [2].

Top Alterations in Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative

Significant Genes in Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative

AFF1 +

CBFB +

CSF3R +

ELL +

IDH1 +

IDH2 +

KMT2A +

MECOM +

MLF1 +

MLLT1 +

MLLT10 +

MLLT3 +

MLLT4 +

MYH11 +

NPM1 +

NRIP3 +

PML +

RARA +

RPN1 +

RUNX1 +

RUNX1T1 +

Disease Details

Synonyms
Subacute Myeloid Leukemia, Subacute Granulocytic Leukemia, Atypical CML, Atypical Chronic Myeloid Leukemia, Subacute Myelogenous Leukemia, aCML
Parent(s)
Myelodysplastic/Myeloproliferative Neoplasm
OncoTree Name
Atypical Chronic Myeloid Leukemia, BCR-ABL1-
OncoTree Code
ACML

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.