ArticleInternational journal of hematology2008
Advanced mast cell disease: an Italian Hematological Multicenter experience.
Article in International journal of hematology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 47 citations in OpenAlex.
- Efficacy and safety of midostaurin in patients with advanced systemic mastocytosis: 10-year median follow-up of a phase II trial.Leukemia · 2018Trial
- Systemic Mastocytosis in Adults: 2026 Update on Diagnosis, Risk Stratification and Management.American journal of hematology · 2026Review
- Genetic Changes in Mastocytes and Their Significance in Mast Cell Tumor Prognosis and Treatment.Genes · 2024Review
- A Review of the Clinical Features and Management of Systemic Congenital Mastocytosis through the Presentation of An Unusual Prenatal-Onset Case.Current oncology (Toronto, Ont.) · 2023Review
- Target Therapies for Systemic Mastocytosis: An Update.Pharmaceuticals (Basel, Switzerland) · 2022Review
- Real-World Efficacy of Midostaurin in Aggressive Systemic Mastocytosis.Journal of clinical medicine · 2021Article
- Mast Cell Activation Disorders.Medicina (Kaunas, Lithuania) · 2021Review
- Recent advances in the understanding and therapeutic management of mastocytosis.F1000Research · 2019Review
- Imatinib in systemic mastocytosis: a phase IV clinical trial in patients lacking exon 17Oncotarget · 2017Article
- Pharmacological treatment options for mast cell activation disease.Naunyn-Schmiedeberg's archives of pharmacology · 2016Review
- Chronic mast cell leukemia (MCL) with KIT S476I: a rare entity defined by leukemic expansion of mature mast cells and absence of organ damage.Annals of hematology · 2015Article
- Chronic mast cell leukemia: a novel leukemia-variant with distinct morphological and clinical features.Leukemia research · 2015Review
- Imatinib: a breakthrough of targeted therapy in cancer.Chemotherapy research and practice · 2014Review
- Mastocytosis: update on pharmacotherapy and future directions.Expert opinion on pharmacotherapy · 2013Review
- Delayed graft-versus-mast-cell effect on systemic mastocytosis with associated clonal haematological non-mast cell lineage disease after allogeneic transplantation.Bone marrow transplantation · 2013Article
- Advanced systemic mastocytosis: the impact of KIT mutations in diagnosis, treatment, and progression.European journal of haematology · 2013Review
- Improved detection of the KIT D816V mutation in patients with systemic mastocytosis using a quantitative and highly sensitive real-time qPCR assay.The Journal of molecular diagnostics : JMD · 2011Article
- [Systemic mastocytosis--definition of an internal disease].Medizinische Klinik (Munich, Germany : 1983) · 2010Review
Corrections and comments
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Authors and funding
24 authors at 17 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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, alpha-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. D816V c-kit mutation is frequent and associated with resistance against Imatinib. Because of the rarity of these forms, an effective standard of care is lacking. More data are needed to find new and successful therapeutic strategies.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.