Outcomes and molecular profile of oligomonocytic CMML support its consideration as the first stage in the CMML continuum

Autor: Xavier Calvo, David Roman-Bravo, Nieves Garcia-Gisbert, Juan Jose Rodriguez-Sevilla, Sara Garcia-Avila, Lourdes Florensa, Joan Gibert, Concepción Fernández-Rodríguez, Marta Salido, Anna Puiggros, Blanca Espinet, Luis Colomo, Beatriz Bellosillo, Ana Ferrer, Leonor Arenillas
Přispěvatelé: Instituto de Salud Carlos III, European Commission, Xarxa de Bancs de Tumors de Catalunya, Calvo, Xavier, García-Gisbert, Nieves, Rodríguez-Sevilla, Juan José, Gibert, Joan, Salido, Marta, Puiggros, Anna, Espinet, Blanca, Colomo, Luis, Bellosillo, Beatriz, Ferrer, Ana, Arenillas, Leonor
Rok vydání: 2022
Předmět:
Zdroj: Blood Advances. 6:3921-3931
ISSN: 2473-9537
2473-9529
DOI: 10.1182/bloodadvances.2022007359
Popis: Patients with oligomonocytic chronic myelomonocytic leukemia (OM-CMML) are currently classified according to the 2017 World Health Organization myelodysplastic syndromes classification. However, recent data support considering OM-CMML as a specific subtype of chronic myelomonocytic leukemia (CMML), given their similar clinical, genomic, and immunophenotypic profiles. The main purpose of our study was to provide survival outcome data of a well-annotated series of 42 patients with OM-CMML and to compare them to 162 patients with CMML, 120 with dysplastic type (D-CMML), and 42 with proliferative type (P-CMML). OM-CMML had significantly longer overall survival (OS) and acute myeloid leukemia-free survival than did patients with CMML, considered as a whole group, and when compared with D-CMML and P-CMML. Moreover, gene mutations associated with increased proliferation (ie, ASXL1 and RAS-pathway mutations) were identified as independent adverse prognostic factors for OS in our series. We found that at a median follow-up of 53.47 months, 29.3% of our patients with OM-CMML progressed to D-CMML, and at a median follow-up of 46.03 months, 28.6% of our D-CMML group progressed to P-CMML. These data support the existence of an evolutionary continuum of OM-CMML, D-CMML, and P-CMML. In this context, we observed that harboring more than 3 mutated genes, carrying ASXL1 mutations, and a peripheral blood monocyte percentage >20% significantly predicted a shorter time of progression of OM-CMML into overt CMML. These variables were also detected as independent adverse prognostic factors for OS in OM-CMML. These data support the consideration of OM-CMML as the first evolutionary stage within the proliferative continuum of CMML.
This study was supported in part by grants from ISCI II-FEDER FIS PI16/0153, FIS PI17/313, FIS PI19/0005, 2017SGR205, and 2017SGR437, and Xarxa de Banc de Tumors de Catalunya.
Databáze: OpenAIRE
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