Mast cell activation syndrome

A new umbrella term for mast cell disease.

Encompasses mastocytosis and mast cell activation syndrome (MCAS),

An episodic symptom related to mast cell mediator release, largely histamine and prostaglandins.

Often presents with chronic multisystem process of inflammatory nature.

Multiple potential triggers including: manual manipulation (Darier sign), surgery, drugs, and sudden temperature changes.

Can aggravate the risk and course of cancers.

MCAS is a relatively nonproliferative disease compared to mastocytosis.

Serum tryptase reflects total body mast cell load, more then the total body mast cell activation state, and is elevated little to none in MCAS as opposed to significant elevations in systemic mastocytosis.

Ab2242ant mess cell mediator release is present in systemic mastocytosis and MCAS, but only in systemic mastocytosis are there aggregates of mast cells in bone marrow and other extracutaneous tissues.

Mast cell activation disease comprises disorders characterized by accumulation of genetically altered mast cells and/or abnormal release of these cells’ mediators, affecting functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing.

In most cases diagnosis is possible by non-invasive testing.

Effective therapy consists of antihistamines and mast cell membrane-stabilizing compounds.

Managed with avoidance of triggers and with anti-mediator therapy, such as H1/H2 blockers, corticosteroids, anti-leukotrienes, and intramuscular epinephrine for severe cases.
Systemic therapies with known activity for systemic mastocyosis include interferon, cladribine, midostaurin, and imatinib.

Mast cell activation disease is considered in the differential diagnosis of patients with chronic multisystem disease.

Mast cell activation disease (MCAD) refers to disorders characterized by accumulation of pathological mast cells in organs and tissues and/or release of mast cell mediators.

Mast cell activation syndrome (MCAS) presents a complex clinical picture of multiple mast cell mediator-induced symptom.

Symptoms observed in patients wi MCAS are little different from those seen in patients systemic mastocytosis.

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