Personalized Management Strategies in Mast Cell Disorders

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