Systemic mastocytosis

Common Name(s)

Systemic mastocytosis

Systemic mastocytosis is a condition caused by the accumulation of mast cells in more than one part of the body. Mast cells contain substances such as histamine that regulate allergic reactions. In systemic mastocytosis, mast cells may build up in the bone marrow, internal organs, or in the skin. This condition is usually diagnosed in adults. Treatment is generally based on a person's symptoms, but it may include antihistamines (to prevent the effect of mast cell histamine) or chemotherapy for more aggressive forms.

There are several different types of systemic mastocytosis. Indolent systemic mastocytosis develops slowly, while aggressive systemic mastocytosis develops more rapidly. Another form, called systemic mastocytosis with associated hemotologic non-mast cell lineage disease (SM-AHNMD), is characterized by the presence of several types of blood disorders. Mast cell leukemia is a rare form of systemic mastocytosis.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Systemic mastocytosis" for support, advocacy or research.

The Mastocytosis Society, Inc

The Mastocytosis Society is a 501(c)3 nonprofit organization dedicated to supporting patients affected by Mastocytosis or Mast Cell Activation Disorders as well as their families, caregivers, and physicians through research, education, and advocacy.

Last Updated: 20 Nov 2012

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Systemic mastocytosis" for support, advocacy or research.

The Mastocytosis Society, Inc

The Mastocytosis Society is a 501(c)3 nonprofit organization dedicated to supporting patients affected by Mastocytosis or Mast Cell Activation Disorders as well as their families, caregivers, and physicians through research, education, and advocacy.

http://www.TMSforacure.org

Last Updated: 20 Nov 2012

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Systemic mastocytosis" returned 170 free, full-text research articles on human participants. First 3 results:

Midostaurin in Advanced Systemic Mastocytosis.
 

Author(s): Marie-Olivia Chandesris, Gandhi Damaj, Danielle Canioni, Chantal Brouzes, Ludovic Lhermitte, Katia Hanssens, Laurent Frenzel, Zoubair Cherquaoui, Isabelle Durieu, Stéphane Durupt, Emmanuel Gyan, Odille Beyne-Rauzy, David Launay, Cyril Faure, Mohamed Hamidou, Sophie Besnard, Momar Diouf, Aurélie Schiffmann, Mathilde Niault, Pierre-Yves Jeandel, Dana Ranta, Remi Gressin, Sylvain Chantepie, Stéphane Barete, Patrice Dubreuil, Philippe Bourget, Olivier Lortholary, Olivier Hermine,

Journal: N. Engl. J. Med.. 2016 Jun;374(26):2605-7.

 

Last Updated: 30 Jun 2016

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Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis.
 

Author(s): Jason Gotlib, Hanneke C Kluin-Nelemans, Tracy I George, Cem Akin, Karl Sotlar, Olivier Hermine, Farrukh T Awan, Elizabeth Hexner, Michael J Mauro, David W Sternberg, Matthieu Villeneuve, Alice Huntsman Labed, Eric J Stanek, Karin Hartmann, Hans-Peter Horny, Peter Valent, Andreas Reiter

Journal: N. Engl. J. Med.. 2016 Jun;374(26):2530-41.

 

Advanced systemic mastocytosis comprises rare hematologic neoplasms that are associated with a poor prognosis and lack effective treatment options. The multikinase inhibitor midostaurin inhibits KIT D816V, a primary driver of disease pathogenesis.

Last Updated: 30 Jun 2016

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The Basophil Activation Test Is Not a Useful Screening Tool for Hymenoptera Venom-Related Anaphylaxis in Patients with Systemic Mastocytosis.
 

Author(s): Mark J A Rietveld, Marco W J Schreurs, Roy Gerth van Wijk, Paul L A van Daele, Maud A W Hermans

Journal: Int. Arch. Allergy Immunol.. 2016 ;169(2):125-9.

 

Systemic mastocytosis (SM) patients are at a high risk for anaphylaxis, with Hymenoptera as the main culprit. A screening instrument to identify which patients are sensitized to Hymenoptera before they experience anaphylaxis would therefore be of great value. The basophil activation ...

Last Updated: 15 Apr 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Systemic mastocytosis" returned 23 free, full-text review articles on human participants. First 3 results:

Critical care management of systemic mastocytosis: when every wasp is a killer bee.
 

Author(s): Hinke Y van der Weide, David J van Westerloo, Walter M van den Bergh

Journal:

 

Since the critical care physician will most likely be involved in a life-threatening expression of systemic mastocytosis, recognition of this disease is of utmost importance in the critical care management of these patients. Mastocytosis is a severely under-recognized disease because ...

Last Updated: 3 Jun 2015

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Systemic mastocytosis in adults: 2015 update on diagnosis, risk stratification, and management.
 

Author(s): Animesh Pardanani

Journal: Am. J. Hematol.. 2015 Mar;90(3):250-62.

 

Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extracutaneous organs.

Last Updated: 18 Feb 2015

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Anaphylactic reaction to platelet transfusion as the initial symptom of an undiagnosed systemic mastocytosis: a case report and review of the literature.
 

Author(s): Clifford R Blieden, German Campuzano-Zuluaga, Adrienne Moul, Jennifer R Chapman, Maureen Cioffi-Lavina, Offiong F Ikpatt, Gerald E Byrne, Francisco Vega

Journal:

 

The association between anaphylactic reactions and systemic mastocytosis is well documented. However, platelet transfusion has not previously been reported as a potential elicitor of anaphylaxis in the context of systemic mastocytosis.

Last Updated: 6 Dec 2014

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Diagnostic Value of Bone Marrow Tryptase in Systemic Mastocytosis
 

Status: Recruiting

Condition Summary: Systemic Mastocytosis

 

Last Updated: 16 Aug 2016

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Cladribine Plus Pegylated Interpheron Alfa-2a in Systemic Mastocytosis
 

Status: Recruiting

Condition Summary: Systemic Mastocytosis

 

Last Updated: 26 Aug 2016

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Brentuximab Vedotin in Treating Patients With Advanced Systemic Mastocytosis or Mast Cell Leukemia
 

Status: Recruiting

Condition Summary: Aggressive Systemic Mastocytosis; Mast Cell Leukemia; Systemic Mastocytosis

 

Last Updated: 20 Nov 2015

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