Development and validation of a prognostic scoring system for patients with chronic myelomonocytic leukemia

Esperanza Such, Ulrich Germing, Luca Malcovati, José Cervera, Andrea Kuendgen, Matteo G. Della Porta, Benet Nomdedeu, Leonor Arenillas, Elisa Luño, Blanca Xicoy, Mari L. Amigo, David Valcarcel, Kathrin Nachtkamp, Ilaria Ambaglio, Barbara Hildebrandt, Ignacio Lorenzo, Mario Cazzola, Guillermo Sanz

Research output: Contribution to journalArticle

Abstract

The natural course of chronic myelomonocytic leukemia (CMML) is highly variable but a widely accepted prognostic scoring system for patients with CMML is not available. The main aim of this study was to develop a new CMML-specific prognostic scoring system (CPSS) in a large series of 558 patients with CMML (training cohort, Spanish Group of Myelodysplastic Syndromes) and to validate it in an independent series of 274 patients (validation cohort, Heinrich Heine University Hospital, Düsseldorf, Germany, and San Matteo Hospital, Pavia, Italy). The most relevant variables for overall survival (OS) and evolution to acute myeloblastic leukemia (AML) were FAB and WHO CMML subtypes, CMML-specific cytogenetic risk classification, and red blood cell (RBC) transfusion dependency. CPSS was able to segregate patients into 4 clearly different risk groups for OS (P <.001) and risk of AML evolution (P <.001) and its predictive capability was confirmed in the validation cohort. An alternative CPSS with hemoglobin instead of RBC transfusion dependency offered almost identical prognostic capability. This study confirms the prognostic impact of FAB and WHO subtypes, recognizes the importance of RBC transfusion dependency and cytogenetics, and offers a simple and powerful CPSS for accurately assessing prognosis and planning therapy in patients with CMML.

Original languageEnglish
Pages (from-to)3005-3015
Number of pages11
JournalBlood
Volume121
Issue number15
DOIs
Publication statusPublished - Apr 11 2013

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

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