Moving towards a uniform risk stratification system in CMML - How far are we?

Best Pract Res Clin Haematol. 2020 Jun;33(2):101131. doi: 10.1016/j.beha.2019.101131. Epub 2019 Dec 6.

Abstract

Many prognostic scoring systems have been developed for chronic myelomonocytic leukemia (CMML). Although these efforts have been informative, no single model has been considered the consensus for CMML prognostication and all models are only moderately prognostic. CMML clinical models utilize mainly hematology and morphology parameters to estimate risk. A better understanding of cytogenetics and the genomic landscape of CMML have resulted in integrated risk models such as CMML Prognostic Scoring System (CPSS)-Mol and Mayo Molecular that may provide better prognostic accuracy for an individual patient. For example, frameshift/nonsense ASXL1 mutations have been consistently shown to confer inferior outcomes leading to its incorporation into some of the major risk classification systems. Prognostication in the setting of therapeutic interventions such as hypomethylating agents and allogeneic hematopoietic cell transplantation have also garnered considerable interest. Despite having many validated risk models available, not a single system is universally adopted. Herein, we will provide an overview of how these systems evolved and progress toward a uniform system.

Keywords: CMML mutations; CMML prognostic scores; CMML risk assessment; Chronic myelomonocytic leukemia.

Publication types

  • Review

MeSH terms

  • Codon, Nonsense*
  • Frameshift Mutation*
  • Humans
  • Leukemia, Myelomonocytic, Chronic* / diagnosis
  • Leukemia, Myelomonocytic, Chronic* / genetics
  • Neoplasm Proteins / genetics*
  • Prognosis
  • Repressor Proteins / genetics*
  • Risk Assessment

Substances

  • ASXL1 protein, human
  • Codon, Nonsense
  • Neoplasm Proteins
  • Repressor Proteins