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

J Natl Compr Canc Netw. 2018 Dec;16(12):1500-1537. doi: 10.6004/jnccn.2018.0088.

Abstract

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.

Publication types

  • Practice Guideline

MeSH terms

  • Anaphylaxis / diagnosis
  • Anaphylaxis / immunology
  • Anaphylaxis / therapy*
  • Antineoplastic Agents / pharmacology
  • Antineoplastic Agents / therapeutic use
  • Biomarkers, Tumor / blood
  • Biomarkers, Tumor / genetics
  • Biopsy
  • Bone Marrow / drug effects
  • Bone Marrow / pathology
  • Hematopoietic Stem Cell Transplantation / methods
  • Hematopoietic Stem Cell Transplantation / standards
  • Histamine Antagonists / pharmacology
  • Histamine Antagonists / therapeutic use
  • Humans
  • Immunophenotyping / methods
  • Immunophenotyping / standards
  • Mast Cells / drug effects
  • Mast Cells / immunology
  • Mast Cells / metabolism
  • Mastocytosis, Systemic / diagnosis
  • Mastocytosis, Systemic / genetics
  • Mastocytosis, Systemic / immunology
  • Mastocytosis, Systemic / therapy*
  • Medical Oncology / methods
  • Medical Oncology / standards*
  • Molecular Targeted Therapy / methods
  • Molecular Targeted Therapy / standards
  • Mutation
  • Oncogene Proteins, Fusion / genetics
  • Patient Care Team / standards*
  • Proto-Oncogene Proteins c-kit / genetics
  • Receptor, Platelet-Derived Growth Factor alpha / genetics
  • Societies, Medical / standards
  • Transplantation, Homologous / methods
  • Transplantation, Homologous / standards
  • Treatment Outcome
  • mRNA Cleavage and Polyadenylation Factors / genetics

Substances

  • Antineoplastic Agents
  • Biomarkers, Tumor
  • Histamine Antagonists
  • Oncogene Proteins, Fusion
  • mRNA Cleavage and Polyadenylation Factors
  • FIP1L1-PDGFRA fusion protein, human
  • KIT protein, human
  • Proto-Oncogene Proteins c-kit
  • Receptor, Platelet-Derived Growth Factor alpha