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

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

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

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

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

Mutational Hotspot of TET2, IDH1, IDH2, SRSF2, SF3B1, KRAS, and NRAS from Human Systemic Mastocytosis Are Not Conserved in Canine Mast Cell Tumors.
 

Author(s): Eleonora Zorzan, Katia Hanssens, Mery Giantin, Mauro Dacasto, Patrice Dubreuil

Journal:

 

Both canine cutaneous mast cell tumor (MCT) and human systemic mastocytosis (SM) are characterized by abnormal proliferation and accumulation of mast cells in tissues and, frequently, by the presence of activating mutations in the receptor tyrosine kinase V-Kit Hardy-Zuckerman 4 Feline ...

Last Updated: 13 Nov 2015

Go To URL
Identification of the Ki-1 antigen (CD30) as a novel therapeutic target in systemic mastocytosis.
 

Author(s): Katharina Blatt, Sabine Cerny-Reiterer, Juliana Schwaab, Karl Sotlar, Gregor Eisenwort, Gabriele Stefanzl, Gregor Hoermann, Matthias Mayerhofer, Mathias Schneeweiss, Sylvia Knapp, Thomas Rülicke, Emir Hadzijusufovic, Karin Bauer, Dubravka Smiljkovic, Michael Willmann, Andreas Reiter, Hans-Peter Horny, Peter Valent

Journal: Blood. 2015 Dec;126(26):2832-41.

 

The Ki-1 antigen (CD30) is an established therapeutic target in patients with Hodgkin lymphoma and anaplastic large-cell lymphoma. We have recently shown that CD30 is expressed abundantly in the cytoplasm of neoplastic mast cells (MCs) in patients with advanced systemic mastocytosis ...

Last Updated: 25 Dec 2015

Go To URL
Nilotinib in patients with systemic mastocytosis: analysis of the phase 2, open-label, single-arm nilotinib registration study.
 

Author(s): Andreas Hochhaus, Michele Baccarani, Francis J Giles, Philipp D le Coutre, Martin C Müller, Andreas Reiter, Helene Santanastasio, Mimi Leung, Steven Novick, Hagop M Kantarjian

Journal: J. Cancer Res. Clin. Oncol.. 2015 Nov;141(11):2047-60.

 

Activating KIT mutations are part of the pathogenesis of systemic mastocytosis (SM). Nilotinib is a tyrosine kinase inhibitor that potently inhibits activated forms of KIT. This phase 2, open-label, single-arm study (CAMN107A2101; www.clinicaltrials.gov NCT00109707) evaluated nilotinib ...

Last Updated: 16 Sep 2015

Go To URL

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 21 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

Go To URL
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

Go To URL
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

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

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

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

Patient-Reported Outcome Questionnaire for Systemic Mastocytosis
 

Status: Recruiting

Condition Summary: Aggressive Systemic Mastocytosis (ASM); Systemic Mastocytosis With Associated Clonal Hematological Non-mast Cell Lineage Disease (SM-AHNMD); Mast Cell Leukemia (MCL); Smoldering Systemic Mastocytosis (SSM); Indolent Systemic Mastocytosis (ISM) [ISM Subgroup Fully Recruited]

 

Last Updated: 2 Mar 2015

Go to URL
Ibrutinib in Treating Patients With Advanced Systemic Mastocytosis
 

Status: Recruiting

Condition Summary: Aggressive Systemic Mastocytosis; Mast Cell Leukemia; Systemic Mastocytosis; Associated Clonal Hematological Non-Mast-Cell Lineage Disease

 

Last Updated: 8 Jan 2016

Go to URL
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

Go to URL