The Spectrum of Mast Cell Activation Syndromes 

Authors: Kelli W. Williams, MD, MPH(1) and Kathleen Luskin, MD

Editor: Kathleen Luskin, MD(2) 

1 Department of Pediatrics, Division of Pediatric Pulmonology, Allergy and  Immunology, and Sleep Medicine, Medical University of South Carolina, Charleston, South Carolina, USA 

2 Allergy/Immunology Department, Scripps Health, San Diego, California, USA 

Corresponding Author: Kelli W. Williams, MD, MPH 

Associate Professor of Pediatrics, Medical University of South Carolina 125 Doughty Street, MSC 917, Suite 530C 

Charleston, SC 29425 

Telephone: (843)-876-1555 

Fax: (843)-876-1583 

Email: williamske@musc.edu 

Keywords: mast cell activation, mastocytosis, hereditary alpha-tryptasemia 

Disclosures (Dr. Kelli Williams): Advisory board/steering committee member for Pharming Healthcare, Amgen, Sumitomo, Kenota Health, Blueprint Medicines; Speaker for Amgen and Pharming Healthcare; Industry sponsored clinical trial principal investigator for GlaxoSmithKline, ADMA Biologics, and Cogent Biosciences 

Table of Contents 

1 Introduction 

2 Diagnostic criteria and classification of mast cell activation syndromes 

2.1 Primary (clonal) mast cell activation syndromes 

2.1.1 Systemic mastocytosis  

2.1.2 Monoclonal mast cell syndrome 

2.2 Secondary (non-clonal) mast cell activation syndromes 

2.3 Idiopathic mast cell conditions 

2.4 Other mast cell disorders 

2.4.1 Hereditary alpha-tryptasemia 

2.4.2 Cutaneous mastocytosis 

2.4.3 Localized mast cell tumors 

3 Summary 

4 References