The aim of the study is to evaluate clinical features, treatments and outcome of patients with systemic mast cell disease (MCD) who arrived to the attention of hematologists. A retrospective study was conducted over 1995–2006 in patients admitted in 18 Italian hematological divisions. Twenty-four cases of advanced MCD were collected: 12 aggressive SM (50%), 8 mast cell leukemia (33%), 4 SM with associated clonal non-mast cell-lineage hematologic disease (17%). Spleen and liver were the principal extramedullary organ involved. The c-kit point mutation D816V was found in 13/18 patients in which molecular biology studies were performed (72%). Treatments were very heterogeneous: on the whole Imatinib was administered in 17 patients, a-Interferon in 8, 2-CdA in 3; 2 patients underwent allogeneic hematopoietic stem cell transplantation. The overall response rate to Imatinib, the most frequently employed drugs, was of 29%, registering one complete remission and four partial remission; all responsive patients did not present D816V c-kit mutation. Overall three patients (12%) died for progression of disease. We conclude that MCD is characterized by severe mediator- related symptoms but with a moderate mortality rate.

Advanced mast cell disease: an Italian Hematological Multicenter experience / Pagano, L; Valentini, Cg; Caira, M; Rondoni, M; VAN LINT, Mt; Candoni, A; Allione, B; Cattaneo, C; Marbello, L; Caramatti, Cecilia; Pogliani, Em; Iannitto, E; Giona, F; Ferrara, F; Invernizzi, R; Fanci, R; Lunghi, M; Fianchi, L; Sanpaolo, G; Stefani, Pm; Pulsoni, A; Martinelli, G; Leone, G; Musto, P.. - In: INTERNATIONAL JOURNAL OF HEMATOLOGY. - ISSN 0925-5710. - 88(5):(2008), pp. 483-488. [10.1007/s12185-008-0166-4]

Advanced mast cell disease: an Italian Hematological Multicenter experience.

CARAMATTI, Cecilia;
2008

Abstract

The aim of the study is to evaluate clinical features, treatments and outcome of patients with systemic mast cell disease (MCD) who arrived to the attention of hematologists. A retrospective study was conducted over 1995–2006 in patients admitted in 18 Italian hematological divisions. Twenty-four cases of advanced MCD were collected: 12 aggressive SM (50%), 8 mast cell leukemia (33%), 4 SM with associated clonal non-mast cell-lineage hematologic disease (17%). Spleen and liver were the principal extramedullary organ involved. The c-kit point mutation D816V was found in 13/18 patients in which molecular biology studies were performed (72%). Treatments were very heterogeneous: on the whole Imatinib was administered in 17 patients, a-Interferon in 8, 2-CdA in 3; 2 patients underwent allogeneic hematopoietic stem cell transplantation. The overall response rate to Imatinib, the most frequently employed drugs, was of 29%, registering one complete remission and four partial remission; all responsive patients did not present D816V c-kit mutation. Overall three patients (12%) died for progression of disease. We conclude that MCD is characterized by severe mediator- related symptoms but with a moderate mortality rate.
Advanced mast cell disease: an Italian Hematological Multicenter experience / Pagano, L; Valentini, Cg; Caira, M; Rondoni, M; VAN LINT, Mt; Candoni, A; Allione, B; Cattaneo, C; Marbello, L; Caramatti, Cecilia; Pogliani, Em; Iannitto, E; Giona, F; Ferrara, F; Invernizzi, R; Fanci, R; Lunghi, M; Fianchi, L; Sanpaolo, G; Stefani, Pm; Pulsoni, A; Martinelli, G; Leone, G; Musto, P.. - In: INTERNATIONAL JOURNAL OF HEMATOLOGY. - ISSN 0925-5710. - 88(5):(2008), pp. 483-488. [10.1007/s12185-008-0166-4]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11381/1867214
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