Back to Search Start Over

Pharmacological treatment options for mast cell activation disease.

Authors :
Molderings GJ
Haenisch B
Brettner S
Homann J
Menzen M
Dumoulin FL
Panse J
Butterfield J
Afrin LB
Source :
Naunyn-Schmiedeberg's archives of pharmacology [Naunyn Schmiedebergs Arch Pharmacol] 2016 Jul; Vol. 389 (7), pp. 671-94. Date of Electronic Publication: 2016 Apr 30.
Publication Year :
2016

Abstract

Mast cell activation disease (MCAD) is a term referring to a heterogeneous group of disorders characterized by aberrant release of variable subsets of mast cell (MC) mediators together with accumulation of either morphologically altered and immunohistochemically identifiable mutated MCs due to MC proliferation (systemic mastocytosis [SM] and MC leukemia [MCL]) or morphologically ordinary MCs due to decreased apoptosis (MC activation syndrome [MCAS] and well-differentiated SM). Clinical signs and symptoms in MCAD vary depending on disease subtype and result from excessive mediator release by MCs and, in aggressive forms, from organ failure related to MC infiltration. In most cases, treatment of MCAD is directed primarily at controlling the symptoms associated with MC mediator release. In advanced forms, such as aggressive SM and MCL, agents targeting MC proliferation such as kinase inhibitors may be provided. Targeted therapies aimed at blocking mutant protein variants and/or downstream signaling pathways are currently being developed. Other targets, such as specific surface antigens expressed on neoplastic MCs, might be considered for the development of future therapies. Since clinicians are often underprepared to evaluate, diagnose, and effectively treat this clinically heterogeneous disease, we seek to familiarize clinicians with MCAD and review current and future treatment approaches.

Details

Language :
English
ISSN :
1432-1912
Volume :
389
Issue :
7
Database :
MEDLINE
Journal :
Naunyn-Schmiedeberg's archives of pharmacology
Publication Type :
Academic Journal
Accession number :
27132234
Full Text :
https://doi.org/10.1007/s00210-016-1247-1