Mastocytosis is a myeloid neoplasm. It is defined by expansion and focal accumulation of clonal mast cells (MCs) in one or more organs.
Mastocytosis has a complex pathology and may be complicated by:
- MC activation
- bone abnormalities
- neurological problems
- gastrointestinal symptoms
- hematologic progression
Mediator-related symptoms can occur in any type of mastocytosis.
The World Health Organization divides mastocytosis into several forms:
- cutaneous
- systemic mastocytosis (SM)
- MC sarcoma
In most patients with SM, somatic mutations in KIT are detected.
Patients with indolent SM have a normal to near-normal life expectancy. This is in contrast with patients with advanced SM, including aggressive SM and MC leukemia, who have a poor prognosis.
Management of patients with SM is an emerging challenge. It requires a multidisciplinary approach.
More in the JACI-IP article below, REMA score and other practical suggestions are listed:
https://www.jaci-inpractice.org/article/S2213-2198(22)00281-1/fulltext
Personalized Management Strategies in Mast Cell Disorders
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