Abstract

SummaryChronic myelomonocytic leukaemia is a rare disease and data on the treatment are often extrapolated from myelodysplastic syndrome studies. Although several scores exist for the prognosis of overall survival in chronic myelomonocytic leukaemia, so far there is no designated score for the prediction of the time to first treatment. We tested clinical parameters and cytogenetic information for their ability to predict the time to first treatment in our single center cohort of 55 unselected consecutive chronic myelomonocytic leukaemia patients. In multivariate analysis we identified elevated lactate dehydrogenase (≥223 U/l), higher bone marrow blast percentage (≥7.5%) and thrombocytopenia (<55 G/l) at initial diagnosis as the most relevant parameters for the time to first treatment. Using these three parameters we developed a risk score that efficiently estimates the time to treatment initiation with azacitidine or hydroxyurea (p < 0.001; log-rank). In the high-risk group (≥2 risk factors) 85% of patients required treatment within 1 year, whereas this was the case in 48% in the intermediate-risk (1 risk factor) and in 0% in the low-risk group (0 risk factors). Our risk model was validated in an external test cohort of 65 patients and may serve as a simplified and easily applicable tool for identifying patients who may not require early treatment initiation.

Highlights

  • Chronic myelomonocytic leukaemia (CMML) is a clonal hematopoietic stem cell disorder that is regarded as myeloproliferative/myelodysplastic overlap disorder according to the 2016 revision to the World Health Organization (WHO) classification of myeloid neoplasms and acute leukaemia

  • According to the FAB classification 58% had myelodysplastic CMML (MD-CMML) and 75% and 25% were categorized as CMML-1 and CMML-2, respectively

  • According to the MD Anderson prognostic score (MDAPS), which is specific for CMML, 26% of our patients were classified as higher-risk (22% intermediate-2, 4% high risk)

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Summary

Introduction

Chronic myelomonocytic leukaemia (CMML) is a clonal hematopoietic stem cell disorder that is regarded as myeloproliferative/myelodysplastic overlap disorder according to the 2016 revision to the World Health Organization (WHO) classification of myeloid neoplasms and acute leukaemia. The Düsseldorf score, international prognostic scoring system (IPSS) and the revised IPSS (IPSS-R) were primarily applied to estimate OS in MDS and included myelodysplastic CMML (MD-CMML) patients [10,11,12]. Molecular abnormalities, such as ASXL1, NRAS, RUNX1 and SETBP1 mutations impact on OS and have already been included in molecular prognostic risk models in CMML [5, 13, 14]

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