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Mastocytosis 2016: Updated WHO Classification and Novel Emerging Treatment Concepts

Peter Valent, Cem Akin and Dean D. Metcalfe

Abstract

Over the past few years substantial advances have been made in understanding the pathogenesis, evolution, and complexity of mast cell neoplasms. New diagnostic and prognostic parameters and novel therapeutic targets with demonstrable clinical impact have been identified. A number of these new markers, molecular targets, and therapeutic approaches have been validated and translated into clinical practice. At the same time, the classification of mastocytosis and related diagnostic criteria have been refined and updated by the consensus group and the World Health Organization (WHO). As a result, more specific therapies tailored towards prognostic sub-groups of patients have been developed. Emerging treatment concepts employ drugs directed against KIT and other relevant targets in neoplastic mast cells, and will hopefully receive recognition by health authorities in the near future. The current article provides an overview of recent developments in the field, with emphasis on the updated WHO classification, refined criteria, additional prognostic parameters, and novel therapeutic approaches. Based on these emerging concepts, the prognosis, quality of life, and survival of patients with advanced mastocytosis are expected to improve in the coming years.

  • Submitted September 2, 2016.
  • Accepted December 17, 2016.