[Indolent systemic mastocytosis : an overview in 2025].

Area of Science:

• Immunology
• Hematology
• Oncology

Background:

• Mast cells (MC) are crucial for immune defense against pathogens and venoms.
• Mastocytosis comprises diseases of abnormal MC accumulation, including cutaneous and systemic forms.

Purpose of the Study:

• To outline the diagnostic criteria and management strategies for indolent systemic mastocytosis (ISM).
• To highlight the increased risk of anaphylaxis in ISM patients and discuss emerging therapeutic options.

Main Methods:

• Diagnosis of ISM relies on identifying MC clusters in bone marrow/extracutaneous organs.
• Key diagnostic markers include elevated serum tryptase, altered MC surface markers, and activating c-KIT mutations.

Main Results:

• Patients with ISM face a heightened risk of severe anaphylaxis, especially from hymenoptera stings.
• Current management involves symptomatic treatment, trigger avoidance, desensitization, and omalizumab.

Conclusions:

• Novel tyrosine kinase inhibitors targeting mutated c-KIT offer promising therapeutic avenues for refractory ISM and advanced mastocytosis.
• Effective management of ISM requires a multi-faceted approach, including risk mitigation and targeted therapies.