Validation of the 2017 revision of the WHO chronic myelomonocytic Leukemia categories

Sanam Loghavi, Dawen Sui, Peng Wei, Guillermo Garcia-Manero, Sherry Pierce, Mark J. Routbort, Elias J. Jabbour, Naveen Pemmaraju, Rashmi Kanagal-Shamanna, H. Deniz Gur, Shimin Hu, Zhuang Zuo, L. Jeffrey Medeiros, Hagop M. Kantarjian, Joseph D. Khoury

Research output: Contribution to journalReview articlepeer-review

31 Scopus citations

Abstract

The 2017 revision of the World Health Organization (WHO) classification includes substantial changes to the subclassification of chronic myelomonocytic leukemia (CMML): (1) a 3-tiered blast-based scheme including a novel “CMML-0” category replacing a 2-tiered system in place since 2001 and (2) 2 CMML subtypes, myelodysplastic (MDS-CMML) and myeloproliferative (MP-CMML), based on a white blood cell count cutoff of 13 3 109/L. The clinical utility of this subclassification scheme, particularly the expansion of blast-based subgroups, has not been validated. In this study, a large single-institution CMML patient cohort (n 5 629) was used to assess the prognostic impact of the newly proposed categories. Patients were risk stratified according to the CMML-specific Prognostic Scoring System (CPSS) and the MD Anderson Prognostic Scoring System. MP-CMML patients had significantly shorter overall survival (OS; P, .0001; hazard ratio: 0.53, 95% confidence interval: 0.42-0.65) and median duration to acute myeloid leukemia (AML) transformation (P, .0001; 15.2 vs 22.0 months) compared with MDS-CMML patients. The CMML-0 group included 36.4% patients with higher risk CPSS categories and 11.2% of patients with high-risk cytogenetics. Among treatment-naïve patients (n 5 499), there was a marginal difference in OS between the CMML-0 and CMML-12017 subgroups (P 5 .0552). The WHO 2017 blast-based categories were not associated with AML-free survival. Incorporation of the WHO 2017 blast-based subgroups in a modified CPSS scheme had a neutral effect and did not improve its prognostic strength. Our data support the inclusion of MP-CMML and MDS-CMML subtypes in the WHO 2017 revision. Although of some utility in MP-CMML, the 3-tiered blast-based system is not well supported in this study.

Original languageEnglish (US)
Pages (from-to)1807-1816
Number of pages10
JournalBlood Advances
Volume2
Issue number15
DOIs
StatePublished - Aug 14 2018
Externally publishedYes

ASJC Scopus subject areas

  • Hematology

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