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

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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A unique presentation of pulmonary disease in advanced systemic mastocytosis, proven by the presence of mast cells in bronchoalveolar lavage: a case report.
 

Author(s): Maud A W Hermans, Annemiek Broijl, Paul L A van Daele

Journal:

 

Systemic mastocytosis is a rare myeloproliferative disease characterized by the uncontrolled proliferation of aberrant mast cells. It has varying clinical manifestations. For unknown reasons, pulmonary localization of mastocytosis is extremely rare.

Last Updated: 14 Oct 2016

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Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension.
 

Author(s): Cláudio Martins, Cristina Teixeira, Suzane Ribeiro, Daniel Trabulo, Cláudia Cardoso, João Mangualde, Ricardo Freire, Élia Gamito, Ana Luísa Alves, Isabelle Cremers, Cecília Alves, Anabela Neves, Ana Paula Oliveira

Journal: World J. Gastroenterol.. 2016 Jul;22(28):6559-64.

 

Mastocytosis is a clonal neoplastic disorder of the mast cells (MC) that can be limited to the skin (cutaneous mastocytosis) or involve one or more extracutaneous organs (systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease ...

Last Updated: 8 Sep 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: 24 Apr 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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Ibrutinib in Treating Patients With Advanced Systemic Mastocytosis
 

Status: Recruiting

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

 

Last Updated: 27 Jun 2017

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