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 172 free, full-text research articles on human participants. First 3 results:

Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management.
 

Author(s): Animesh Pardanani

Journal: Am. J. Hematol.. 2016 Nov;91(11):1146-1159.

 

Disease overview:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extra-cutaneous organs.

Last Updated: 20 Oct 2016

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Risk of solid cancer, cardiovascular disease, anaphylaxis, osteoporosis and fractures in patients with systemic mastocytosis: A nationwide population-based study.
 

Author(s): Sigurd Broesby-Olsen, Dóra Körmendiné Farkas, Hanne Vestergaard, Anne Pernille Hermann, Michael Boe Møller, Charlotte Gotthard Mortz, Thomas Kielsgaard Kristensen, Carsten Bindslev-Jensen, Henrik Toft Sørensen, Henrik Frederiksen

Journal: Am. J. Hematol.. 2016 Nov;91(11):1069-1075.

 

In patients with systemic mastocytosis (SM), several aspects of morbidity remain poorly understood. We assessed the risk of solid cancers, cardiovascular disease, anaphylaxis, osteoporosis, and fractures in SM patients. Using Danish medical registries, we conducted a nationwide population-based ...

Last Updated: 4 Aug 2016

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Well-differentiated systemic mastocytosis showed excellent clinical response to imatinib in the absence of known molecular genetic abnormalities: A case report.
 

Author(s): Lanshan Huang, Sa A Wang, Sergej Konoplev, Carlos E Bueso-Ramos, Beenu Thakral, Roberto N Miranda, Elias Jabbour, L Jeffrey Medeiros, Rashmi Kanagal-Shamanna

Journal: Medicine (Baltimore). 2016 Oct;95(41):e4934.

 

Well-differentiated systemic mastocytosis (WDSM) is a rare, recently recognized provisional subvariant of systemic mastocytosis (SM). We report a case of WDSM that showed excellent clinical and cutaneous response to imatinib in the absence of known molecular genetic abnormalities.

Last Updated: 14 Oct 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 24 free, full-text review articles on human participants. First 3 results:

Advanced systemic mastocytosis: from molecular and genetic progress to clinical practice.
 

Author(s): Celalettin Ustun, Michel Arock, Hanneke C Kluin-Nelemans, Andreas Reiter, Wolfgang R Sperr, Tracy George, Hans-Peter Horny, Karin Hartmann, Karl Sotlar, Gandhi Damaj, Olivier Hermine, Srdan Verstovsek, Dean D Metcalfe, Jason Gotlib, Cem Akin, Peter Valent

Journal: Haematologica. 2016 Oct;101(10):1133-1143.

 

Systemic mastocytosis is a heterogeneous disease characterized by the accumulation of neoplastic mast cells in the bone marrow and other organ organs/tissues. Mutations in KIT, most frequently KIT D816V, are detected in over 80% of all systemic mastocytosis patients. While most systemic ...

Last Updated: 3 Oct 2016

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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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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: 25 Oct 2017

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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: 27 Jun 2017

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