Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology
Open accessAbstract
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.
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- Authors
- Gotlib, J., Gerds, A. T., Bose, P., Castells, M. C., Deininger, M. W., Gojo, I., Gundabolu, K., Hobbs, G., Jamieson, C., McMahon, B., Mohan, S. R., Oehler, V., Oh, S., Padron, E., Pancari, P., Papadantonakis, N., Pardanani, A., Podoltsev, N., Rampal, R., Ranheim, E., Rein, L., Snyder, D. S., Stein, B. L., Talpaz, M., Thota, S., Wadleigh, M., Walsh, K., Bergman, M. A., & Sundar, H.
- Affiliations
- Memorial Sloan Kettering Cancer Center (US)
- Barnes-Jewish Hospital (US)
- Stanford Cancer Institute (US)
- University Hospitals Seidman Cancer Center (US)
- University of Alabama at Birmingham (US)
- ORCID
- Gotlib, Jason
- Gerds, Aaron T.
- Bose, Prithviraj
- Castells, Mariana C.
- Deininger, Michael W.
- Gojo, Ivana
- Gundabolu, Krishna
- Hobbs, Gabriela
- Jamieson, Catriona
- McMahon, Brandon
- Mohan, Sanjay R.
- Oehler, Vivian
- Oh, Stephen
- Padron, Eric
- Papadantonakis, Nikolaos
- Pardanani, Animesh
- Podoltsev, Nikolai
- Rampal, Raajit
- Ranheim, Erik
- Rein, Lindsay
- Stein, Brady L.
- Talpaz, Moshe
- Thota, Swapna
- Wadleigh, Martha
- Venue
- Journal of the National Comprehensive Cancer Network
- Publisher
- Harborside Press, LLC
- Published
- 2018-12
- Volume
- 16
- Issue
- 12
- Pages
- 1500-1537
- ISSN
- 1540-1405 (ISSN-L)
- 1540-1413
- OpenAlex
- W2904923610
- Cited by
- 106
- References
- 144
- Citations by year
- 2026: 3 · 2025: 3 · 2024: 4 · 2023: 4 · 2022: 10 · 2021: 24 · 2020: 33 · 2019: 22 · 2018: 3
- FWCI
- 8.80
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of the National Comprehensive Cancer Network (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal Tumor Research and Treatment
- Health Sciences › Medicine › Physiology › Histiocytic Disorders and Treatments
- Keywords
- systemic mastocytosis, mast cells, KIT D816V mutation, targeted therapy, diagnosis, skin involvement
- MeSH terms
- Anaphylaxis, Antineoplastic Agents, Biopsy, Bone Marrow, Histamine Antagonists, Humans, Mast Cells, Medical Oncology, Mutation, Patient Care Team, Societies, Medical, Transplantation, Homologous, Biomarkers, Tumor, Oncogene Proteins, Fusion, Immunophenotyping, Treatment Outcome, Hematopoietic Stem Cell Transplantation, Proto-Oncogene Proteins c-kit, Receptor, Platelet-Derived Growth Factor alpha, Mastocytosis, Systemic, mRNA Cleavage and Polyadenylation Factors, Molecular Targeted Therapy
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