The Italian Mastocytosis Registry: 6-year experience from a hospital-based registry

Author:

Merante Serena12,Ferretti Virginia V1,Elena Chiara1,Brazzelli Valeria3,Zanotti Roberta4,Neri Iria5,Magliacane Diomira6,Belloni Fortina Anna7,Ingeborg Forer8,Pastorello Elide A9,Pieri Lisa10,Papayannidis Cristina11,Mauro Marina12,Grifoni Federica13,Minelli Roberto14,Guggiari Elena15,Difonzo Elisa16,Bocchia Monica17,Caroppo Francesca7,Di Nuzzo Sergio18,Elli Elena Maria19,Rondoni Michela20,Ciccocioppo Rachele21,Di Stefano Michele22,Bossi Grazia23,Boveri Emanuela24,Bonadonna Patrizia25,Giona Fiorina26,Valent Peter2728,Triggiani Massimo6

Affiliation:

1. Hematology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

2. Hematology Department, James Cook University Hospital, South Tees Trust (NHS), Middlesbrough, UK

3. Dermatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

4. Hematology, Policlinico GB Rossi, Verona, Italy

5. Dermatology, Department of Experimental, Diagnostic & Specialty Medicine, University of Bologna, Bologna, Italy

6. Immunology & Allergology, University of Salerno, Salerno, Italy

7. Pediatric Dermatology, DIMED, University of Padua, Padua, Italy

8. Dermatololgy & Venerology, Hospital of Brunico, Italy

9. Allergology & Immunology, Niguarda Hospital, Milano, Italy

10. Center of Research & Innovation of Myeloproliferative Neoplasms (CRIMM), Careggi University Hospital/University of Florence, Florence, Italy

11. Hematology, University of Bologna, Bologna, Italy

12. Allergy Unit, Sant'Anna Hospital, Como, Italy

13. Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy

14. Pediatric, University of Cagliari, Cagliari, Italy

15. Hematology, San Raffaele Hospital, Milano, Italy

16. Dermatology, University of Florence, Florence, Italy

17. Hematology, University of Siena, Siena, Italy

18. Dermatology, University of Parma, Parma, Italy

19. Divisione di Ematologia, Ospedale San Gerardo, ASST Monza, Italy

20. Hematology, Ospedale di Cesena, Cesena, Italy

21. Gastroenterology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy

22. Internal Medicine, IRCCS, Policlinico San Matteo, Pavia, Italy

23. Pediatric Department, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

24. Pathology Department, Fondazione IRCCS Policlinico San Matteo, Pavia, University of Pavia, Italy

25. Allergology, Ospedale Civile Maggiore, Verona, Italy

26. Ematologia, Sapienza Università di Roma, Azienda Policlinico Umberto I Roma, Italy

27. Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria

28. Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria

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.

Publisher

Future Medicine Ltd

Subject

Cancer Research,Oncology,General Medicine

Cited by 11 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Systemic Mastocytosis in Adults: Long-Term Follow up and Real-Life Experience;International Journal of Hematology and Oncology;2024-06-30

2. Cutaneous mastocytosis and infantile hemangioma: is it only a coincidence?;Italian Journal of Dermatology and Venereology;2022-02

3. Olfactory Function Is Impaired in Patients with Mastocytosis;The Journal of Allergy and Clinical Immunology: In Practice;2021-03

4. Mastocytosis;Skin and the Heart;2021

5. Mastocytosis in Adults: A Retrospective Analysis of the Clinical Course and Treatment of 58 Patients;Clinical oncohematology;2021

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