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Ann Oncol. 2014 Sep;25(9):1691-700. doi: 10.1093/annonc/mdu047. Epub 2014 Mar 27.

Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML): a consensus proposal.

Author information

  • 1Division of Hematology, Department of Medicine I, Medical University of Vienna, Vienna, Austria
  • 2Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany.
  • 3Division of Hematology, Department of Medicine I, Medical University of Vienna, Vienna, Austria.
  • 4Servicio Central de Citometria, Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) and Department of Medicine, University of Salamanca, Salamanca, Spain.
  • 5Division of Hematology, Department of Internal Medicine, University of Istanbul, Turkey.
  • 6III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
  • 7Department of Pathology, University of New Mexico, Albuquerque, USA.
  • 8Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
  • 9Imagine Institute Université Paris Descartes, Sorbonne, Paris Cité, Centre national de référence des mastocytoses, Paris, France.
  • 10Division of Allergic Diseases, Mayo Clinic, Rochester, USA.
  • 11Hematology Center Karolinska, Karolinska University Hospital, Stockholm, Sweden.
  • 12Division of Hematology-Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis.
  • 13Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, USA.
  • 14Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
  • 15Department of Haematology, Guys and St Thomas' NHS Foundation Trust, Guys Hospital, London, UK.
  • 16Division of Allergy and Immunology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, USA.
  • 17Department of Dermatology, University of Cologne, Cologne, Germany.
  • 18Stanford Cancer Center, Stanford University School of Medicine, Stanford.
  • 19Department of Internal Medicine, Division of Rheumatology, Allergy & Immunology, Virginia Commonwealth University, Richmond.
  • 20Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston.
  • 21Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, USA.
  • 22LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France.


Mast cell leukemia (MCL), the leukemic manifestation of systemic mastocytosis (SM), is characterized by leukemic expansion of immature mast cells (MCs) in the bone marrow (BM) and other internal organs; and a poor prognosis. In a subset of patients, circulating MCs are detectable. A major differential diagnosis to MCL is myelomastocytic leukemia (MML). Although criteria for both MCL and MML have been published, several questions remain concerning terminologies and subvariants. To discuss open issues, the EU/US-consensus group and the European Competence Network on Mastocytosis (ECNM) launched a series of meetings and workshops in 2011-2013. Resulting discussions and outcomes are provided in this article. The group recommends that MML be recognized as a distinct condition defined by mastocytic differentiation in advanced myeloid neoplasms without evidence of SM. The group also proposes that MCL be divided into acute MCL and chronic MCL, based on the presence or absence of C-Findings. In addition, a primary (de novo) form of MCL should be separated from secondary MCL that typically develops in the presence of a known antecedent MC neoplasm, usually aggressive SM (ASM) or MC sarcoma. For MCL, an imminent prephase is also proposed. This prephase represents ASM with rapid progression and 5%-19% MCs in BM smears, which is generally accepted to be of prognostic significance. We recommend that this condition be termed ASM in transformation to MCL (ASM-t). The refined classification of MCL fits within and extends the current WHO classification; and should improve prognostication and patient selection in practice as well as in clinical trials.


KIT D816V; leukemia; mast cells; mastocytosis; prognostication; tryptase

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