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

Adult mastocytosis: a review of the Santo António Hospital 's experience and an evaluation of World Health Organization criteria for the diagnosis of systemic disease.
 

Author(s): Iolanda Conde Fernandes, Maria dos Anjos Teixeira, Inês Freitas, Manuela Selores, Rosário Alves, Margarida Lima

Journal: An Bras Dermatol. ;89(1):59-66.

 

Mastocytosis is a clonal disorder characterized by the accumulation of abnormal mast cells in the skin and/or in extracutaneous organs.

Last Updated: 14 Mar 2014

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A clinicopathologic study of 24 cases of systemic mastocytosis involving the gastrointestinal tract and assessment of mucosal mast cell density in irritable bowel syndrome and asymptomatic patients.
 

Author(s): Leona A Doyle, Golrokh J Sepehr, Matthew J Hamilton, Cem Akin, Mariana C Castells, Jason L Hornick

Journal: Am. J. Surg. Pathol.. 2014 Jun;38(6):832-43.

 

Counting mast cells in gastrointestinal (GI) mucosal biopsies is becoming an increasingly common practice. The primary reason for this exercise is to evaluate for possible involvement by systemic mastocytosis (SM). However, the features of mastocytosis in GI biopsies are not well ...

Last Updated: 13 May 2014

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Systemic mastocytosis with associated myeloproliferative neoplasm with t(8;19)(p12;q13.1) and abnormality of FGFR1: report of a unique case.
 

Author(s): Christina B Duckworth, Linsheng Zhang, Shiyong Li

Journal:

 

Systemic mastocytosis is a neoplastic proliferation of mast cells that frequently presents with associated clonal hematological non-mast cell lineage disease. Myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene are a heterogenous group of rare and aggressive hematopoietic ...

Last Updated: 19 Feb 2014

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

Indolent systemic mastocytosis: a case and review of the current available treatment options.
 

Author(s): Yvonne Lee, Peter Wood, H Peter Soyer

Journal:

 

Mastocytosis denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells. Cutaneous mastocytosis is characterized by skin-limited disease and is the most common subtype. Systemic mastocytosis has extracutaneous organ involvement with ...

Last Updated: 9 Sep 2013

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Indolent systemic mastocytosis limited to the bone: a case report and review of the literature.
 

Author(s): Pedro Pinto-Lopes, Francisco Adao Fonseca, Roberto Silva, Pedro von Hafe, Elsa Fonseca

Journal: Sao Paulo Med J. 2013 ;131(3):198-204.

 

CONTEXT Systemic mastocytosis is defined as a clonal disorder of mast cells and their precursor cells and is currently classified as a myeloproliferative neoplasm. Its clinical course has a wide spectrum, ranging from indolent disease, with normal life expectancy, to highly aggressive ...

Last Updated: 1 Aug 2013

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International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) & European Competence Network on Mastocytosis (ECNM) consensus response criteria in advanced systemic mastocytosis.
 

Author(s): Jason Gotlib, Animesh Pardanani, Cem Akin, Andreas Reiter, Tracy George, Olivier Hermine, Hanneke Kluin-Nelemans, Karin Hartmann, Wolfgang R Sperr, Knut Brockow, Lawrence B Schwartz, Alberto Orfao, Daniel J Deangelo, Michel Arock, Karl Sotlar, Hans-Peter Horny, Dean D Metcalfe, Luis Escribano, Srdan Verstovsek, Ayalew Tefferi, Peter Valent

Journal: Blood. 2013 Mar;121(13):2393-401.

 

Systemic mastocytosis (SM) is characterized by accumulation of neoplastic mast cells and is classified into indolent and aggressive forms. The latter include aggressive SM (ASM), mast cell leukemia (MCL), and SM associated with a myeloid neoplasm wherein 1 or both disease compartments ...

Last Updated: 29 Mar 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

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Clinical Trial Information This information is provided by ClinicalTrials.gov

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: 17 Dec 2013

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Use of Tamoxifen in Systemic Mastocytosis
 

Status: Recruiting

Condition Summary: Systemic Mastocytosis

 

Last Updated: 11 Aug 2014

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Midostaurin in Indolent Systemic Mastocytosis
 

Status: Recruiting

Condition Summary: Indolent Systemic Mastocytosis

 

Last Updated: 11 Oct 2013

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