Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts

被引：502

作者：

Valent, Peter ^{[1
,2
]}

Akin, Cem ^{[3
]}

Metcalfe, Dean D. ^{[4
]}

机构：

[1] Med Univ Vienna, Dept Med 1, Div Hematol & Hemostaseol, Waehringer Guertel 18-20, Vienna, Austria

[2] Med Univ Vienna, Dept Med 1, Ludwig Boltzmann Cluster Oncol, Vienna, Austria

[3] Harvard Med Sch, Brigham & Womens Hosp, Div Allergy & Immunol, Dept Med, Boston, MA USA

[4] NIAID, Mast Cell Biol Sect, Lab Allerg Dis, NIH, 9000 Rockville Pike, Bethesda, MD 20892 USA

来源：

BLOOD | 2017年 / 129卷 / 11期

基金：

奥地利科学基金会; 美国国家卫生研究院;

关键词：

ADVANCED SYSTEMIC MASTOCYTOSIS; NEOPLASTIC MAST-CELLS; CURRENT TREATMENT OPTIONS; KINASE INHIBITOR PKC412; C-KIT MUTATION; OF-THE-ART; THERAPEUTIC TARGET; RESPONSE CRITERIA; GEMTUZUMAB OZOGAMICIN; URTICARIA PIGMENTOSA;

D O I：

10.1182/blood-2016-09-731893

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1420 / 1427

页数：8

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