Advances in the classification and treatment of mastocytosis: Current status and outlook toward the future

Peter Valent*, Cem Akin, Karin Hartmann, Gunnar Nilsson, Andreas Reiter, Olivier Hermine, Karl Sotlar, Wolfgang R Sperr, Luis Escribano, Tracy I George, Hanneke C Kluin-Nelemans, Celalettin Ustun, Massimo Triggiani, Knut Brockow, Jason Gotlib, Alberto Orfao, Lawrence B Schwartz, Sigurd Broesby-Olsen, Carsten Bindslev-Jensen, Petri T. KovanenStephen J. Galli, K. Frank Austen, Daniel A. Arber, Hans-Peter Horny, Michel Arock, Dean D Metcalfe

*Corresponding author for this work

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Abstract

Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and its basic concept remains valid. However, refinements have recently been proposed by the consensus group, the WHO, and the European Competence Network on Mastocytosis. In addition, new treatment options are available for patients with advanced systemic mastocytosis, including allogeneic hematopoietic stem cell transplantation and multikinase inhibitors directed against KIT D816V and other key signaling molecules. Our current article provides an overview of recent advances in the field of mastocytosis, with emphasis on classification, prognostication, and emerging new treatment options in advanced systemic mastocytosis.

Original languageEnglish
JournalCancer Research
Volume77
Issue number6
Pages (from-to)1261-1270
ISSN0008-5472
DOIs
Publication statusPublished - 15. Mar 2017

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Systemic Mastocytosis
Mastocytosis
Mast Cells
Mast-Cell Leukemia
Cutaneous Mastocytosis
Mental Competency
Consensus

Keywords

  • Disease Progression
  • Humans
  • Mastocytosis/classification

Cite this

Valent, P., Akin, C., Hartmann, K., Nilsson, G., Reiter, A., Hermine, O., ... Metcalfe, D. D. (2017). Advances in the classification and treatment of mastocytosis: Current status and outlook toward the future. Cancer Research, 77(6), 1261-1270. https://doi.org/10.1158/0008-5472.CAN-16-2234
Valent, Peter ; Akin, Cem ; Hartmann, Karin ; Nilsson, Gunnar ; Reiter, Andreas ; Hermine, Olivier ; Sotlar, Karl ; Sperr, Wolfgang R ; Escribano, Luis ; George, Tracy I ; Kluin-Nelemans, Hanneke C ; Ustun, Celalettin ; Triggiani, Massimo ; Brockow, Knut ; Gotlib, Jason ; Orfao, Alberto ; Schwartz, Lawrence B ; Broesby-Olsen, Sigurd ; Bindslev-Jensen, Carsten ; Kovanen, Petri T. ; Galli, Stephen J. ; Austen, K. Frank ; Arber, Daniel A. ; Horny, Hans-Peter ; Arock, Michel ; Metcalfe, Dean D. / Advances in the classification and treatment of mastocytosis : Current status and outlook toward the future. In: Cancer Research. 2017 ; Vol. 77, No. 6. pp. 1261-1270.
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abstract = "Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and its basic concept remains valid. However, refinements have recently been proposed by the consensus group, the WHO, and the European Competence Network on Mastocytosis. In addition, new treatment options are available for patients with advanced systemic mastocytosis, including allogeneic hematopoietic stem cell transplantation and multikinase inhibitors directed against KIT D816V and other key signaling molecules. Our current article provides an overview of recent advances in the field of mastocytosis, with emphasis on classification, prognostication, and emerging new treatment options in advanced systemic mastocytosis.",
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Valent, P, Akin, C, Hartmann, K, Nilsson, G, Reiter, A, Hermine, O, Sotlar, K, Sperr, WR, Escribano, L, George, TI, Kluin-Nelemans, HC, Ustun, C, Triggiani, M, Brockow, K, Gotlib, J, Orfao, A, Schwartz, LB, Broesby-Olsen, S, Bindslev-Jensen, C, Kovanen, PT, Galli, SJ, Austen, KF, Arber, DA, Horny, H-P, Arock, M & Metcalfe, DD 2017, 'Advances in the classification and treatment of mastocytosis: Current status and outlook toward the future', Cancer Research, vol. 77, no. 6, pp. 1261-1270. https://doi.org/10.1158/0008-5472.CAN-16-2234

Advances in the classification and treatment of mastocytosis : Current status and outlook toward the future. / Valent, Peter; Akin, Cem; Hartmann, Karin; Nilsson, Gunnar; Reiter, Andreas; Hermine, Olivier; Sotlar, Karl; Sperr, Wolfgang R; Escribano, Luis; George, Tracy I; Kluin-Nelemans, Hanneke C; Ustun, Celalettin; Triggiani, Massimo; Brockow, Knut; Gotlib, Jason; Orfao, Alberto; Schwartz, Lawrence B; Broesby-Olsen, Sigurd; Bindslev-Jensen, Carsten; Kovanen, Petri T.; Galli, Stephen J.; Austen, K. Frank; Arber, Daniel A.; Horny, Hans-Peter; Arock, Michel; Metcalfe, Dean D.

In: Cancer Research, Vol. 77, No. 6, 15.03.2017, p. 1261-1270.

Research output: Contribution to journalReviewResearchpeer-review

TY - JOUR

T1 - Advances in the classification and treatment of mastocytosis

T2 - Current status and outlook toward the future

AU - Valent, Peter

AU - Akin, Cem

AU - Hartmann, Karin

AU - Nilsson, Gunnar

AU - Reiter, Andreas

AU - Hermine, Olivier

AU - Sotlar, Karl

AU - Sperr, Wolfgang R

AU - Escribano, Luis

AU - George, Tracy I

AU - Kluin-Nelemans, Hanneke C

AU - Ustun, Celalettin

AU - Triggiani, Massimo

AU - Brockow, Knut

AU - Gotlib, Jason

AU - Orfao, Alberto

AU - Schwartz, Lawrence B

AU - Broesby-Olsen, Sigurd

AU - Bindslev-Jensen, Carsten

AU - Kovanen, Petri T.

AU - Galli, Stephen J.

AU - Austen, K. Frank

AU - Arber, Daniel A.

AU - Horny, Hans-Peter

AU - Arock, Michel

AU - Metcalfe, Dean D

PY - 2017/3/15

Y1 - 2017/3/15

N2 - Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and its basic concept remains valid. However, refinements have recently been proposed by the consensus group, the WHO, and the European Competence Network on Mastocytosis. In addition, new treatment options are available for patients with advanced systemic mastocytosis, including allogeneic hematopoietic stem cell transplantation and multikinase inhibitors directed against KIT D816V and other key signaling molecules. Our current article provides an overview of recent advances in the field of mastocytosis, with emphasis on classification, prognostication, and emerging new treatment options in advanced systemic mastocytosis.

AB - Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and its basic concept remains valid. However, refinements have recently been proposed by the consensus group, the WHO, and the European Competence Network on Mastocytosis. In addition, new treatment options are available for patients with advanced systemic mastocytosis, including allogeneic hematopoietic stem cell transplantation and multikinase inhibitors directed against KIT D816V and other key signaling molecules. Our current article provides an overview of recent advances in the field of mastocytosis, with emphasis on classification, prognostication, and emerging new treatment options in advanced systemic mastocytosis.

KW - Disease Progression

KW - Humans

KW - Mastocytosis/classification

U2 - 10.1158/0008-5472.CAN-16-2234

DO - 10.1158/0008-5472.CAN-16-2234

M3 - Review

C2 - 28254862

AN - SCOPUS:85015959575

VL - 77

SP - 1261

EP - 1270

JO - Cancer Research

JF - Cancer Research

SN - 0008-5472

IS - 6

ER -