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EnglishThe 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.
Pagano, L., Valentini, C. G., Caira, M., Advanced mast cell disease: an Italian Hematological Multicenter experience., <<INTERNATIONAL JOURNAL OF HEMATOLOGY>>, 2008; (Dicembre): 483-488 [http://hdl.handle.net/10807/3834]
| Autori: | |
| Titolo: | Advanced mast cell disease: an Italian Hematological Multicenter experience. |
| Data di pubblicazione: | 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. |
| Lingua: | Inglese |
| Rivista: | |
| Citazione: | Pagano, L., Valentini, C. G., Caira, M., Advanced mast cell disease: an Italian Hematological Multicenter experience., <<INTERNATIONAL JOURNAL OF HEMATOLOGY>>, 2008; (Dicembre): 483-488 [http://hdl.handle.net/10807/3834] |
| Appare nelle tipologie: | Articolo in rivista, Nota a sentenza |
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