
Is It Systemic Mastocytosis?
Best Practices for Diagnosis and Management
Meeting Slides

Program Handout
A Quick Guide to Systemic Mastocytosis
Clinical Practice Guidelines
The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.
Arber DA, Orazi A, Hasserjian R, et al. Blood. 2016;127(20):2391-2405.
NCCN Clinical Practice Guidelines in Oncology: Systemic Mastocytosis.
National Comprehensive Cancer Network.
Patient Resources
The Mast Cell Disease Society, Inc. (TMS)
TMS is dedicated to providing multifaceted support to patients, families, and medical professionals in the community and to leading the advancement of knowledge and research in mast cell diseases through education, advocacy, and collaboration.
The European Competence Network on Mastocytosis (ECNM)
The goal of the ECNM is to improve disease-recognition, diagnosis, and therapy in patients with mastocytosis in Europe.
Genetic and Rare Diseases Information Center (GARD)
GARD helps patients find information, services, experts, financial aid, and support groups.
National Organization for Rare Disorders (NORD)
NORD is a patient advocacy organization committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and patient services.
Suggested Readings
Results from Pioneer: a randomized, double-blind, placebo-controlled, phase 2 study of avapritinib in patients with indolent systemic mastocytosis.
Akin C, Elberink HO, Gotlib J, et al. American Society of Hematology Virtual Conference; December 5-8, 2020. Abstract 1248.
Update on mastocytosis (part 1): pathophysiology, clinical features, and diagnosis.
Azaña JM, Torrelo A, Matito A. Actas Dermosifiliogr. 2016;107(1):5-14.
Patients with mast cell activation symptoms and elevated baseline serum tryptase level have unique bone marrow morphology.
Giannetti MP, Akin C, Hufdhi R, et al. J Allergy Clin Immunol. 2021;147(4):1497-1501.e1491.
Patient perceptions in mast cell disorders
Jennings SV, Slee VM, Zack RM, et al. Immunol Allergy Clin N Am. 2018;38(3):505-525.
Catching the clinical and biological diversity for an appropriate therapeutic approach in systemic mastocytosis.
Mannelli F. Ann Hematol. 2021;100(2):337-344.
Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management.
Pardanani A. Am J Hematol. 2021;96(4):508-525.
How we diagnose and treat systemic mastocytosis in adults.
Scherber RM, Borate U. Br J Haematol. 2018;180(1):11-23.
Mast cells, mastocytosis, and related disorders.
Theoharides TC, Valent P, Akin C. N Engl J Med 2015;373(2):163-172.
Mastocytosis: lessons learned from the 2019 Society for Hematopathology/European Association for Haematopathology Workshop.
Tzankov A, Duncavage E, Craig FE, et al. Am J Clin Pathol. 2021;155(2):239-266.
Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification.
Trizuljak J, Sperr WR, Nekvindova L, et al. Allergy. 2020;75(8):1923-1934.
Bone marrow mastocytosis: a diagnostic challenge.
Zanotti R, Tanasi I, Bernardelli A, et al. J Clin Med. 2021;10(7):1420.

Insights into Pathophysiologic and Clinical Elements of Systemic Mastocytosis
Presented by Tracy I. George, MD
Listen to Barbara share her story about being diagnosed and living with indolent SM
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