ArticleAllergy2020
Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification.
Article in Allergy, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed, 74 citations in OpenAlex.
- KIT mutation and AHN-associated oncogenic profiles in systemic mastocytosis with an associated hematological neoplasm.Blood advances · 2026Article
- Systemic Mastocytosis in Adults: 2026 Update on Diagnosis, Risk Stratification and Management.American journal of hematology · 2026Review
- Diagnosing Systemic Mastocytosis: State of the Art.International journal of laboratory hematology · 2026Review
- Management of skeletal-related events and fracture prevention in systemic mastocytosis.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2026Review
- Advances in the Management of Mediator-Related Symptoms in Non-Advanced Systemic Mastocytosis.ImmunoTargets and therapy · 2026Review
- Gender and Allergy: Mechanisms, Clinical Phenotypes, and Therapeutic Response-A Position Paper from the Società Italiana di Allergologia, Asma ed Immunologia Clinica (SIAAIC).International journal of molecular sciences · 2025Review
- The evaluation, management, and future of indolent systemic mastocytosis.Annals of hematology · 2025Review
- Understanding Cutaneous Mastocytosis: A Case Report on the Symptoms and Diagnosis of a Rare Disease.Cureus · 2025Article
- Real-world characteristics of systemic mastocytosis in Romania: insights from a reference-center-based descriptive study.Journal of medicine and life · 2025Observational
- Systemic mastocytosis: current status and challenges in 2024.Blood advances · 2025Review
- Mastocytosis.Nature reviews. Disease primers · 2025Review
- Utility of tryptase genotyping in the screening, diagnosis, and management of systemic mastocytosis.Frontiers in allergy · 2025Review
- Prevalence and incidence of mastocytosis in adults: a Danish nationwide register study.European journal of epidemiology · 2025Article
- MASTering systemic mastocytosis: Lessons learned from a large patient cohort.The journal of allergy and clinical immunology. Global · 2024Article
- Article
- [Mastocytosis-a frequently unrecognized disease].Dermatologie (Heidelberg, Germany) · 2024Article
- Review
- Recent advances in diagnosis and therapy in systemic mastocytosis.Blood research · 2023Review
- Secretory and Membrane-Associated Biomarkers of Mast Cell Activation and Proliferation.International journal of molecular sciences · 2023Review
- Comprehensive mastocytosis data analysis from a single center.BMC cancer · 2023Article
Corrections and comments
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Authors and funding
43 authors at 20 institutions in 12 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIn indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM.
methodsWe employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM.
resultsWe found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly.
conclusionOur data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.
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