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Current Oncology Reports

, 21:101 | Cite as

Chronic Myelomonocytic Leukemia: Insights into Biology, Prognostic Factors, and Treatment

  • Giacomo Coltro
  • Mrinal M. PatnaikEmail author
Leukemia (A Aguayo, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Leukemia

Abstract

Purpose of Review

Chronic myelomonocytic leukemia (CMML) is a clonal hematological malignancy characterized by both dysplastic and proliferative features, with an inherent risk for leukemic transformation. With the help of this review, we aim to summarize key concepts with regards to CMML biology, diagnosis, risk stratification, and therapeutics.

Recent Findings

Based on recent studies, CMML is hallmarked by a relatively low genetic complexity, which contrasts with a compelling phenotypical heterogeneity, largely driven by epigenetic mechanisms. Recent advances in the characterization of CMML biology has led to an improvement in risk-stratification, by means of incorporating prognostically relevant gene mutations. This, however, has not significantly impacted available therapies and outcomes continue to remain poor.

Summary

Advances in CMML biology have better explained the phenotypic heterogeneity, while continuing to define the genetic and epigenetic landscape. In spite of recent advances, limited effective therapies exist and developing rationally derived therapeutic approaches is much needed.

Keywords

Chronic myelomonocytic leukemia Myelodysplastic syndromes Myeloproliferative neoplasms Clonal architecture Prognostication Target therapy 

Abbreviations

ASXL1

Additional sex combs like 1

BAP1

BRCA1-associated protein 1

BCOR

BCL6 corepressor

CBL

Cbl proto-oncogene

CSF3R

Colony-stimulating factor 3 receptor

DNMT3A

DNA methyltransferase 3 alpha

EZH2

Enhancer of zeste 2 polycomb repressive complex 2 subunit

FLT3

Fms-related tyrosine kinase 3

IDH1/IDH2

Isocitrate dehydrogenase 1/2

JAK2

Janus kinase 2

NPM1

Nucleophosmin 1

NRAS

Neuroblastoma RAS viral oncogene homolog

PHF6

Plant homeodomain finger protein 6

PRC1/2

Polycomb repressive complex 1/2

PTPN11

Protein tyrosine phosphatase non-receptor type 11

RUNX1

Runt-related transcription factor 1

SETBP1

SET binding protein 1

SF3B1

Splicing factor 3b subunit 1

SRSF2

Serine and arginine rich splicing factor 2

TET2

Tet methylcytosine dioxygenase 2

TP53

Tumor protein P53

U2AF1

U2 small nuclear RNA auxiliary factor 1

SPRY2

Sprouty RTK signaling antagonist 2

US FDA

United States Food and Drug Administration

ZRSR2

Zinc finger CCCH-type RNA binding motif and serine/arginine rich 2

Notes

Compliance with Ethical Standards

Conflict of Interest

Giacomo Coltro declares that he has no conflict of interest.

Mrinal M. Patnaik served on an advisory board for Stem Line Pharmaceuticals. The honorarium was issued directly to Mayo Clinic.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Supplementary material

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of MedicineDivision of Hematology, Mayo ClinicRochesterUSA

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