Current treatment options in patients with mastocytosis: status in 2015 and future perspectives

European Journal of Haematology
Michel ArockPeter Valent

Abstract

Mastocytosis is a term referring to a heterogeneous group of disorders characterized by abnormal mast cell (MC) accumulation in the skin and/or internal organs. In children, the disease involves mostly the skin (cutaneous mastocytosis; CM), whereas in adults, the disease is usually systemic (systemic mastocytosis; SM). Advanced SM variants with end-organ damage and reduced life expectancy have also been described, but are rare. Clinical signs and symptoms in SM result from excessive mediator release by MCs and, in aggressive forms, from organ failure related to MC infiltration. As a consequence, treatment of indolent SM aims primarily at the control of symptoms caused by MC mediator release. By contrast, in advanced SM, such as aggressive SM, MC leukemia, and MC sarcoma, intensive (chemo)therapy with or without allogeneic stem cell transplantation has to be considered. In addition, activating mutations in KIT (mostly KIT D816V in adults) are found in most patients with SM, so that targeted therapies aimed at blocking mutant KIT variants or/and 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 futur...Continue Reading

References

Feb 27, 1992·The New England Journal of Medicine·H C Kluin-NelemansR Willemze
May 1, 1990·The Journal of Allergy and Clinical Immunology·R F HoranK F Austen
Jan 1, 1987·International Archives of Allergy and Applied Immunology·E GomezR J Davies
May 1, 1989·The Journal of Allergy and Clinical Immunology·B V KettelhutD D Metcalfe
Jan 7, 1998·Clinical Pediatrics·M R Lester, D L Bratton
Feb 24, 2001·The New England Journal of Medicine·A TefferiH C Hoagland
May 30, 2001·Leukemia Research·P ValentJ M Bennett
Aug 10, 2001·American Journal of Epidemiology·J S Kaufman, R S Cooper
Mar 29, 2002·International Archives of Allergy and Immunology·Alexandra S Worobec, Dean D Metcalfe
Dec 11, 2002·British Journal of Haematology·Philippe CasassusOlivier Lortholary
Aug 23, 2003·Blood·Hanneke C Kluin-NelemansRob Fijnheer
Apr 15, 2006·Inflammation & Allergy Drug Targets·Luis EscribanoLawrence B Schwartz
May 10, 2006·Clinical Chemistry and Laboratory Medicine : CCLM·Ahmed Salem AliYrjö T Konttinen
Jul 11, 2006·Current Opinion in Allergy and Clinical Immunology·Franziska RuëffBernhard Przybilla
Aug 26, 2006·Immunology and Allergy Clinics of North America·Mariana Castells
Nov 1, 2006·Allergology International : Official Journal of the Japanese Society of Allergology·Hirohisa SaitoYoshimichi Okayama
Jan 30, 2007·Laboratory Investigation; a Journal of Technical Methods and Pathology·Nami Nakagomi, Seiichi Hirota
Sep 27, 2007·Annals of Oncology : Official Journal of the European Society for Medical Oncology·A Olivieri, L Manzione
Sep 18, 2007·Advances in Immunology·Gunnar PejlerSara Wernersson
Nov 21, 2007·Journal of Dermatological Science·Stephen J Galli, Mindy Tsai
Jan 8, 2008·The Journal of Allergy and Clinical Immunology·David González de OlanoLuis Escribano
Feb 21, 2008·European Journal of Haematology·Duncan PurtillPaul Cannell
May 3, 2008·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Michael C HeinrichUNKNOWN Imatinib Target Exploration Consortium Study B2225
May 27, 2008·Clinical Pediatrics·Laura D Briley, Charles M Phillips
Jun 19, 2008·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Srdan VerstovsekHagop Kantarjian
Jul 16, 2008·International Archives of Allergy and Immunology·Joseph H Butterfield, Catherine R Weiler
Aug 8, 2008·Blood·Dean D Metcalfe

❮ Previous
Next ❯

Citations

May 2, 2016·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Celalettin UstunPeter Valent
Feb 10, 2016·Current Allergy and Asthma Reports·Phil Lieberman, Lene Heise Garvey
Aug 5, 2017·Frontiers in Medicine·Mélanie VaesOlivier Hermine
Mar 3, 2020·Frontiers in Immunology·Deniz RagipogluVerena Fischer
Dec 12, 2019·F1000Research·Julien RossignolOlivier Hermine
Apr 26, 2017·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Iolanda Conde-FernandesMargarida Lima
Dec 13, 2016·Current Treatment Options in Allergy·Merel C OnnesJoanne N G Oude Elberink
Jul 21, 2017·Clinical Reviews in Allergy & Immunology·Daniel Elieh Ali KomiStefan Wöhrl
Oct 12, 2020·The Australasian Journal of Dermatology·Cosimo Di RaimondoLuca Bianchi
Oct 17, 2020·Expert Opinion on Biological Therapy·Jason GotlibAndreas Reiter
Apr 4, 2021·Journal of Clinical Medicine·Aneta Szudy-SzczyrekMarek Hus
Oct 14, 2021·Acta Dermato-venereologica·Dominika Kwiatkowska, Adam Reich

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

© 2022 Meta ULC. All rights reserved