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systemic mastocytosis treatment

Systemic Mastocytosis Treatment

Systemic mastocytosis (SM) is a heterogeneous condition that results from multifocal infiltration of clonally derived mast cells (MCs) in cutaneous and extracutaneous tissues. The abnormal MCs usually contain an activating mutation at codon 816 of the KIT gene. The most frequent mutation is the KITD816V mutation, detectable in >80% of all SM patients and in…

SM patient education

Systemic Mastocytosis Patient Education

Systemic mastocytosis is a rare blood disease that is caused by too many mast cells, a type of white blood cell, being produced in the bone marrow. This disease is rarely inherited. It is usually acquired due to genetic mutations.1  Because the body produces too many mast cells, they accumulate in abnormal quantities in the…

Systemic mastocytosis comorbidities

Systemic Mastocytosis Comorbidities

Systemic mastocytosis (SM) is a condition in which mast cells proliferate uncontrollably and accumulate in 1 or more extracutaneous organs. The most typically implicated extracutaneous location is the bone marrow, and bone marrow aspiration and biopsy are frequently used to confirm the diagnosis. Patients with SM have a wide range of clinical outcomes and prognoses,…

systemic mastocytosis prognosis

Systemic Mastocytosis Prognosis

Systemic mastocytosis (SM) is a progressive neoplastic disorder in which adult patients have a shorter life expectancy than that of the general population. In general, most deaths occur within the first 3 to 5 years after diagnosis. However, the prognosis of patients with SM varies due to the variable clinical phenotype, which ranges from indolent…

SM Epidemiology

Systemic Mastocytosis Epidemiology

Systemic mastocytosis is a rare hematological disease characterized by the overproliferation of mast cells, which are white blood cells produced in the bone marrow, within the connective tissues throughout multiple organ systems, including the liver, spleen, skin, bones, lungs, joints, and digestive tract.1,2 Upon activation by an allergen, mast cells release inflammatory chemical mediators such…

systemic mastocytosis guidelines

Systemic Mastocytosis Guidelines

The National Comprehensive Cancer Network (NCCN) has developed a set of guidelines and recommendations for the diagnosis and management of systemic mastocytosis (SM).1 The guidelines are based on the latest research and the recommendations of specialists from NCCN cancer centers with years of experience in treating SM. A diagnostic algorithm is included in the guidelines,…

systemic mastocytosis diagnosis

Systemic Mastocytosis Diagnosis

Systemic mastocytosis is a rare hematological disease characterized by excessive proliferation of mast cells, a type of white blood cell produced in the bone marrow, throughout the body’s tissues and organs, including the skin, liver, lungs, digestive tract, bones, joints, and nerves.1 Mast cells contain chemical inflammatory mediators such as histamine, heparin, growth factors, and…

Systemic Mastocytosis Complications

Systemic Mastocytosis Complications

Mastocytosis comprises a group of diseases in which pathologic mast cells proliferate and accumulate in the tissues in excessive amounts. Systemic mastocytosis (SM), specifically, is a severe form of mastocytosis marked by the release of many vasoactive cell mediators as a result of excessive mast cell activity, which can cause a wide range of symptoms.1 …

systemic mastocytosis therapies

Systemic Mastocytosis Therapies

Mastocytosis is a heterogeneous disorder characterized by the abnormal growth and accumulation of morphologically and immunophenotypically abnormal mast cells (MCs) in 1 or more organs. The systemic form of the disease, systemic mastocytosis (SM), is characterized by diffuse and/or focal infiltration of neoplastic MCs in various organs including the bone marrow, spleen, liver, and gastrointestinal…

systemic mastocytosis testing

Systemic Mastocytosis Testing

The World Health Organization (WHO) has outlined the criteria for the diagnosis of systemic mastocytosis (SM). The diagnosis of SM can be established by the presence of either 1 major and 1 minor criterion or 3 minor criteria.1 Major criterion: The presence of multifocal dense infiltrates of mast cells (MCs) (≥15 MCs in aggregates) in…