Universität zu Köln

Valent, P., Sotlar, K., Sperr, W. R., Escribano, L., Yavuz, S., Reiter, A., George, T. I., Kluin-Nelemans, H. C., Hermine, O., Butterfield, J. H., Hagglund, H., Ustun, C., Hornick, J. L., Triggiani, M., Radia, D., Akin, C., Hartmann, K., Gotlib, J., Schwartz, L. B., Verstovsek, S., Orfao, A., Metcalfe, D. D., Arock, M. and Horny, H. -P. (2014). Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML): a consensus proposal. Ann. Oncol., 25 (9). S. 1691 - 1701. AMSTERDAM: ELSEVIER. ISSN 1569-8041

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Abstract

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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Valent, P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sotlar, K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sperr, W. R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Escribano, L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Yavuz, S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Reiter, A. UNSPECIFIED UNSPECIFIED UNSPECIFIED George, T. I. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kluin-Nelemans, H. C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hermine, O. UNSPECIFIED UNSPECIFIED UNSPECIFIED Butterfield, J. H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hagglund, H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Ustun, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hornick, J. L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Triggiani, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Radia, D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Akin, C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hartmann, K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Gotlib, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schwartz, L. B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Verstovsek, S. UNSPECIFIED UNSPECIFIED UNSPECIFIED Orfao, A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Metcalfe, D. D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Arock, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Horny, H. -P. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-430997
DOI:	10.1093/annonc/mdu047
Journal or Publication Title:	Ann. Oncol.
Volume:	25
Number:	9
Page Range:	S. 1691 - 1701
Date:	2014
Publisher:	ELSEVIER
Place of Publication:	AMSTERDAM
ISSN:	1569-8041
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ACUTE MYELOID-LEUKEMIA; AGGRESSIVE SYSTEMIC MASTOCYTOSIS; IMMUNOPHENOTYPIC ANALYSIS; KIT MUTATIONS; DIFFERENTIATION; EXPRESSION; DISEASE; DELINEATION; DISORDERS; VARIANTS Multiple languages Oncology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/43099