The Italian Mastocytosis Registry: 6-year experience from a Hospital-Based Registry

Abstract

Aim: We collected ‘real-life’ data on the management of patients with mastocytosis in the Italian Mastocytosis Registry. Methods: Six hundred patients diagnosed with mastocytosis between 1974 and 2014 were included from 19 centers. Results: Among adults (n = 401); 156 (38.9%) patients were diagnosed with systemic mastocytosis. In 212 adults, no bone marrow studies were performed resulting in a provisional diagnosis of mastocytosis of the skin. This diagnosis was most frequently established in nonhematologic centers. In total, 182/184 pediatric patients had cutaneous mastocytosis. We confirmed that in the most patients with systemic mastocytosis, serum tryptase levels were >20 ng/ml and KIT D816V was detectable. Conclusion: The Italian Mastocytosis Registry revealed some center-specific approaches for diagnosis and therapy. Epidemiological evidence on this condition is provided.

Keywords::

• hospital-based registry
• mastocytosis
• multidisciplinary management

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Supplementary data

To view the supplementary data that accompany this paper please visit the journal website at: https://www.tandfonline.com/doi/suppl/10.2217/fon-2018-0291

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

The authors would like to acknowledge the work of L Giacomelli and A Shah, who provided medical writing and editorial assistance; this assistance was supported by internal funds.

Ethical conduct of research

The authors state that they have obtained appropriate institutional review board approval or have followed the principles outlined in the Declaration of Helsinki for all human or animal experimental investigations. In addition, for investigations involving human subjects, informed consent has been obtained from the participants involved. The protocol for each study was approved by the appropriate ethics committees and all patients gave informed consent for study participation.