Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML)

A consensus proposal

P. Valent, K. Sotlar, W. R. Sperr, L. Escribano, S. Yavuz, A. Reiter, T. I. George, H. C. Kluin-Nelemans, O. Hermine, J. H. Butterfield, H. Hägglund, C. Ustun, J. L. Hornick, M. Triggiani, D. Radia, C. Akin, K. Hartmann, J. Gotlib, L. B. Schwartz, S. Verstovsek & 4 others A. Orfao, D. D. Metcalfe, M. Arock, H. P. Horny

Research output: Contribution to journalReview article

50 Citations (Scopus)

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) formofMCL 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 currentWHO classification; and should improve prognostication and patient selection in practice as well as in clinical trials.

Original languageEnglish (US)
Article numbermdu047
Pages (from-to)1691-1700
Number of pages10
JournalAnnals of Oncology
Volume25
Issue number9
DOIs
StatePublished - Jan 1 2014

Fingerprint

Mast-Cell Leukemia
Consensus
Leukemia
Systemic Mastocytosis
Mast Cells
Mast-Cell Sarcoma
Bone Marrow
Mastocytosis
Terminology
Mental Competency
Patient Selection
Neoplasms

Keywords

  • KIT D816V
  • Leukemia
  • Mast cells
  • Mastocytosis
  • Prognostication
  • Tryptase

Cite this

Valent, P., Sotlar, K., Sperr, W. R., Escribano, L., Yavuz, S., Reiter, A., ... Horny, H. P. (2014). Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML): A consensus proposal. Annals of Oncology, 25(9), 1691-1700. [mdu047]. https://doi.org/10.1093/annonc/mdu047

Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML) : A consensus proposal. / 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.; Hägglund, 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.; Horny, H. P.

In: Annals of Oncology, Vol. 25, No. 9, mdu047, 01.01.2014, p. 1691-1700.

Research output: Contribution to journalReview article

Valent, P, Sotlar, K, Sperr, WR, Escribano, L, Yavuz, S, Reiter, A, George, TI, Kluin-Nelemans, HC, Hermine, O, Butterfield, JH, Hägglund, H, Ustun, C, Hornick, JL, Triggiani, M, Radia, D, Akin, C, Hartmann, K, Gotlib, J, Schwartz, LB, Verstovsek, S, Orfao, A, Metcalfe, DD, Arock, M & Horny, HP 2014, 'Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML): A consensus proposal', Annals of Oncology, vol. 25, no. 9, mdu047, pp. 1691-1700. https://doi.org/10.1093/annonc/mdu047
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. ; Hägglund, 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. ; Horny, H. P. / Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML) : A consensus proposal. In: Annals of Oncology. 2014 ; Vol. 25, No. 9. pp. 1691-1700.
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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) formofMCL 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 currentWHO classification; and should improve prognostication and patient selection in practice as well as in clinical trials.",
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AU - Valent, P.

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AU - Escribano, L.

AU - Yavuz, S.

AU - Reiter, A.

AU - George, T. I.

AU - Kluin-Nelemans, H. C.

AU - Hermine, O.

AU - Butterfield, J. H.

AU - Hägglund, H.

AU - Ustun, C.

AU - Hornick, J. L.

AU - Triggiani, M.

AU - Radia, D.

AU - Akin, C.

AU - Hartmann, K.

AU - Gotlib, J.

AU - Schwartz, L. B.

AU - Verstovsek, S.

AU - Orfao, A.

AU - Metcalfe, D. D.

AU - Arock, M.

AU - Horny, H. P.

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N2 - 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) formofMCL 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 currentWHO classification; and should improve prognostication and patient selection in practice as well as in clinical trials.

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