A case report of systemic mastocytosis associated with multiple hematologic non–mast cell lineage diseases. Issue 2 (4th April 2019)

• Record Type: Journal Article
• Authors: Grifoni, Federica Irene; Sciumè, Mariarita; Pravettoni, Valerio; Ulivieri, Fabio Massimo; Muratori, Simona; Fracchiolla, Nicola Stefano; Tagliaferri, Elena; Gianelli, Umberto; Migliorini, Anna Chiara; Cro, Lilla; Pacilli, Annalisa; Mannelli, Francesco; Baldini, Luca
• Is Part Of: Hematological oncology. Volume 37:Issue 2(2019)
• Page Start: 205
• Page End: 211
• Publication Date: 2019-04-04
• Subjects: associated hematologic non–mast cell lineage disease -- midostaurin -- systemic mastocytosis; Hematological oncology -- Periodicals; Hematology; Medical Oncology; 616.99418005
• DOI: 10.1002/hon.2605
• Languages: English
• ISSNs: 0278-0232

Abstract

Abstract: Systemic mastocytosis (SM) is a hematological malignancy characterized by extracutaneous infiltration by atypical mast cells. Together with indolent SM, aggressive SM, and mast cell leukemia, the World Health Organization (WHO) recognizes another major disease subgroup: SM with an associated hematological neoplasm, which is characterized by the presence of a concurrent neoplasm, more commonly, a chronic myelomonocytic leukemia. While KIT D816V is commonly regarded as the driver mutation, the clinical presentation of SM is extremely varied. Treatment of SM might not be simple, but now more specific therapies tailored toward prognostic subgroups of patients have been developed. Here, we report a detailed description of clinical management and biological features of a systemic mastocytocis case associated with multiple hematologic non–mast cell lineage diseases.