Haematologica. 2019 May 2. pii: haematol.2019.222059. doi: 10.3324/haematol.2019.222059 |
Proposed diagnostic criteria for classical CMML, CMML variants and pre-CMML conditions. |
Valent P1, Orazi A2, Savona MR3, Patnaik MM4, Onida F5, van de Loosdrecht AA6, Haase D7, Haferlach T8, Elena C9, Pleyer L10, Kern W11, Pemovska T12, Vladimer GI13, Schanz J14, Keller A15, Lubbert M16, Lion T17, Sotlar K18, Reiter A19, De Witte T20, Pfeilstocker M21, Geissler K22, Padron E23, Deininger M24, Orfao A25, Horny HP26, Greenberg PL27, Arber DA28, Malcovati L29, Bennett JM30 |
Abstract Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by dysplasia, abnormal production and accumulation of monocytic cells and an elevated risk to transform into acute leukemia. Over the past two decades, our knowledge about the pathogenesis and molecular mechanisms in CMML has increased substantially. In parallel, better diagnostic criteria and therapeutic strategies have been developed. However, many questions remain regarding prognostication and optimal therapy. In addition, there is a need to define potential pre-phases of CMML and special CMML variants, and to separate these entities from each other and from conditions mimicking CMML. To address these unmet needs, an international consensus group met in a Working Conference in August 2018 and discussed open questions and issues around CMML, its variants, and pre-CMML conditions. The outcomes of this meeting are summarized herein and include diagnostic criteria and a proposed classification of pre-CMML conditions as well as refined minimal diagnostic criteria for classical CMML and special CMML variants, including oligomonocytic CMML and CMML associated with systemic mastocytosis. Moreover, we propose diagnostic standards and tools to delineate between normal, pre-CMML and CMML entities. These criteria and standards should facilitate diagnostic and prognostic evaluations in daily practice and clinical studies in applied hematology. |
Copyright © 2019, Ferrata Storti Foundation. |
KEYWORDS: Acute Myeloid Leukemia, Adult Acute Lymphoblastic Leukemia, Hodgkin's Lymphoma |
Publikations ID: 31048353 Quelle: öffnen |