Internal medicine

Systemic mastocytosis

Family medicine

Oncology

Dermatology

Immunology

Mast cell

Clinical Practice

Cutaneous Mastocytosis

Medicine

Journal of the National Comprehensive Cancer Network

Mastocytosis is a group of heterogeneous disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin and/or in various extracutaneous organs. Systemic mastocytosis is the most common form of mastocytosis diagnosed in adults, characterized by mast cell infiltration of one or more extracutaneous organs (with or without skin involvement). The identification of KIT D816V mutation and the emergence of novel targeted therapies have significantly improved the diagnosis and treatment of systemic mastocytosis. However, certain aspects of clinical care, particularly the diagnosis, assessment, and management of mediator-related symptoms continue to present challenges. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of patients with systemic mastocytosis.

Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology