Successful treatment of idiopathic mast cell activation syndrome with low-dose omalizumab
Open accessAbstract
OBJECTIVES: Idiopathic mast cell disorders, a recently defined and recognised syndrome in clinical practice, are similar to the previously termed non-clonal mast cell disorder. Patients with idiopathic mast cell activation syndrome (MCAS) suffer all the classical signs of mast cell activation but do not have evidence of mast cell clonality. Furthermore, treatment of these patients can be limited and burdensome in those with refractory symptoms. METHODS: Here, we describe treatment of a patient with idiopathic MCAS utilising a single monthly subcutaneous injection of omalizumab and review the current classification and therapeutic options for clonal and non-clonal MCAS. RESULTS: Low-dose omalizumab treatment has successfully led to a 5-year, sustained clinical response, controlled debilitating symptoms of mast cell activation and allowed for reintroduction and long-term maintenance of bee venom subcutaneous immunotherapy. CONCLUSION: Low-dose omalizumab of 150 mg monthly should be considered for maintenance management of patients with idiopathic MCAS for its cost and quality-of-life benefits.
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Response to additional drugs, including multitargeted tyrosine kinase inhibitors (in patients with SM, such as midostaurin) and anti-IgE therapy (omalizumab), may be considered an indirect sign of MCA or MCAS.
“Response to additional drugs, including multitargeted tyrosine kinase inhibitors (in patients with SM, such as midostaurin) and anti-IgE therapy (ie, omalizumab) may be considered an indirect sign of MCA or MCAS.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [85]
The Comments section (page not provided) treats improvement with omalizumab as evidence of recurrent mast cell activation in a patient with idiopathic MCAS, supporting the anti-IgE portion of the claim. The paper does not address responses to multitargeted tyrosine kinase inhibitors such as midostaurin in patients with systemic mastocytosis. (LLM, conf=0.88)
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- Authors
- Berry, R., Hollingsworth, P., & Lucas, M.
- Affiliations
- Sir Charles Gairdner Hospital (AU)
- The University of Western Australia (AU)
- Corresponding author
- Lucas, Michaela
- Venue
- Clinical & Translational Immunology
- Publisher
- Wiley
- Published
- 2019-01
- Volume
- 8
- Issue
- 10
- Pages
- e01075-e01075
- ISSN
- 2050-0068 (ISSN-L)
- PMC
- PMC6768441
- OpenAlex
- W2978342824
- Cited by
- 15
- References
- 10
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 3 · 2023: 2 · 2021: 5 · 2020: 2
- FWCI
- 0.70
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Clinical & Translational Immunology (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- UWA Profiles and Research Repository (UWA) (submittedVersion, cc-by)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- UWA Profiles and Research Repository (University of Western Australia) (submittedVersion, cc-by)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Immunology and Allergy › Food Allergy and Anaphylaxis Research
- Health Sciences › Medicine › Physiology › Asthma and respiratory diseases
- Keywords
- omalizumab, mast cell activation, subcutaneous immunotherapy, bee venom immunotherapy, maintenance therapy, refractory symptoms, quality of life
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