Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts.

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. Several 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 toward prognostic subgroups of patients have been developed. Emerging treatment concepts use drugs directed against KIT and other relevant targets in neoplastic mast cells and will hopefully receive recognition by health authorities in the near future. This 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.

journal_name

Blood

journal_title

Blood

authors

Valent P,Akin C,Metcalfe DD

doi

10.1182/blood-2016-09-731893

subject

Has Abstract

pub_date

2017-03-16 00:00:00

pages

1420-1427

issue

11

eissn

0006-4971

issn

1528-0020

pii

blood-2016-09-731893

journal_volume

129

pub_type

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