Suggested Readings

Tyrosine kinase inhibitors in non-advanced systemic mastocytosis.

Akin C. Immunol Allergy Clin N Am. 2023;43:743–750.

Management of Bone Health in Adult Mastocytosis

Degboé Y, Nezzar C, Alary P, et al. Curr Osteoporos Rep. 2025;23(1):10.

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.e1.

Avapritinib versus placebo in indolent systemic mastocytosis.

Gotlib J, Castells M, Elberink HO, et al. NEJM. 2023;2(6):EVIDoa2200339.

Patient-reported outcomes among patients with systemic mastocytosis in routine clinical practice: results of the TouchStone SM patient survey.

Mesa RA, Sullivan EM, Dubinski D, et al. Cancer. 2022;128(20):3691-3699.

Systemic mastocytosis in adults: 2023 update on diagnosis, risk stratification and management.

Pardanani A. Am J Hematol. 2023;98:1097-1116. 

Mast cells, mastocytosis, and related disorders.

Theoharides TC, Valent P, Akin C. N Engl J Med. 2015;373(2):163-172.

Personalized management strategies in mast cell disorders: ECNM-AIM user's guide for daily clinical practice.

Valent P, Hartmann K, Schwaab J, et al. J Allergy Clin Immunol. 2022;10(8):1999-2012.e1996.

Systemic mastocytosis: multidisciplinary approach.

Zanotti R, Tanasi I, Crosera L, et al. Mediterr J Hematol Infect Dis. 2021;13(1):e2021068.

Clinical Guidelines and Resources

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.

The 5th edition of the World Health Organization classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms.

Khoury JD, Solary E, Abla O, et al. Leukemia. 2022;36(7):1703-1719.

International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data.

Arber DA, Orazi A, Hasserjian RP, et al. Blood. 2022;140(11):1200-1228.

NCCN Clinical Practice Guidelines in Oncology: Systemic Mastocytosis.

National Comprehensive Cancer Network.

Total Rise in Peripheral Tryptase After Systemic Event (TRIPTASE) Calculator

American Initiative in Mast Cell Diseases (AIM)

AIM is dedicated to advance the research, education, and treatment of mastocytosis and related mast cell diseases.

Linked Resources

Video Library

Pathophysiology and Differential Diagnosis of Nonadvanced SM

Dr. Matthew Giannetti reviews the pathophysiology of nonadvanced systemic mastocytosis (SM), emphasizing the role of KIT mutations and the use of high-sensitivity assays for accurate detection. Dr. Giannetti also addresses subtype classification, hereditary alpha tryptasemia (HαT), and key considerations for distinguishing SM from conditions with similar presentations in clinical practice, to support accurate and timely diagnosis.

Individualized Treatment Strategies for Nonadvanced SM

Dr. Thanai Pongdee reviews treatment approaches for nonadvanced systemic mastocytosis (SM), including antihistamines, leukotriene inhibitors, and the tyrosine kinase inhibitor avapritinib. Dr. Pongdee also provides practical considerations for incorporating avapritinib into the treatment paradigm, including dosing, safety, and monitoring guidance, to personalize therapy and improve symptom control.

Patient Education and Support for Nonadvanced SM

Dr. Matthew Giannetti discusses strategies for patient education and support in nonadvanced systemic mastocytosis (SM), focusing on trigger avoidance, periprocedural precautions, and anaphylaxis management. Dr. Giannetti also shares guidance on managing medication triggers and connecting patients with advocacy resources to improve safety and enhance quality of life.

Multidisciplinary Management and Monitoring of Patients With Nonadvanced SM

Dr. Thanai Pongdee highlights the importance of multidisciplinary collaboration for comprehensive management of patients with nonadvanced systemic mastocytosis (SM). Dr. Pongdee also provides practical recommendations on patient monitoring, including laboratory and imaging assessments as well as symptom burden tracking, to guide long-term management and optimize patient outcomes.

Downloadable Clinician and Patient Handouts

Diagnostic Checklist

This handout has been formatted to print on an 8.5x11 sheet of paper. You can choose to print it on 2 separate pages, or double-sided by adjusting your print settings to print on both sides of the paper along the long edge (individual printer settings may vary). For assistance with printing, please contact cmaster@integritasgrp.com

Clinician Pocket Guide

This handout has been formatted to print on an 8.5x11 sheet of paper. In your printer's settings, you should print on both sides of the paper along the short edge (individual printer settings may vary). You should fold it accordion-style, as shown here. For assistance with printing/folding, please contact cmaster@integritasgrp.com

Patient Wallet Card

This handout has been formatted to print 2 wallet cards on an 8.5x11 sheet of paper. To separate the two cards after printing, and to better fit inside a wallet, trim off the white border and along the center line. You can fold it accordion-style, as shown here. For assistance with printing/folding, please contact cmaster@integritasgrp.com

Patient Videos

Indolent Systemic Mastocytosis (ISM): The Patient Experience

Listen to Barbara, Laura, and Mary Jane share their stories about being diagnosed and living with indolent SM.

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 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.

NCCN Guidelines for Patients: Systemic Mastocytosis.

National Comprehensive Cancer Network.

 

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