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PEDIATRIC MASTOCYTOSIS: AN UPDATE
Mastocytosis is a rare clonal disorder, characterized by excessive proliferation and accumulation of mast cells (MC) in various organs and tissues. Cutaneous mastocytosis (CM), the most common form in children, is defined when MC infiltration is limited ...
Fiorina Giona
doaj +6 more sources
Safety and efficacy of avapritinib in advanced systemic mastocytosis: the phase 1 EXPLORER trial
Advanced systemic mastocytosis (AdvSM) is a rare hematologic neoplasm driven by the KIT D816V mutation and associated with poor survival. This phase 1 study (NCT02561988) evaluated avapritinib (BLU-285), a selective KIT D816V inhibitor, in patients with ...
Daniel J Deangelo +2 more
exaly +2 more sources
Advanced systemic mastocytosis (AdvSM) is a rare, KIT D816V-driven hematologic neoplasm characterized by mast cell infiltration and shortened survival.
Jason Gotlib +2 more
exaly +2 more sources
French guidelines for the management of nonadvanced mastocytosis in adults [PDF]
Mastocytosis is a rare disease caused by abnormal mast cell accumulation/proliferation in various organs/tissues. Its clinical features exhibit significant variability irrespective of its clinical presentation, classification, global survival or impact ...
Cristina Bulai Livideanu +5 more
doaj +2 more sources
Epidemiology of mastocytosis: a population-based study (Sweden)
Background: Mastocytosis is a disease characterized by accumulation of aberrant mast cells and mediator-related symptoms and is divided into systemic mastocytosis (SM) and cutaneous mastocytosis (CM).
Anna Bergström +4 more
doaj +2 more sources
Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management
Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in extra‐cutaneous organs.
Animesh D Pardanani
exaly +2 more sources
Osteoporosis and other manifestations of bone disease are frequent in patients with systemic mastocytosis (SM) in association with the presence of mast cell infiltrates in bone marrow, although the mechanisms behind bone disease remain poorly understood.
Do-Kyun Kim +2 more
exaly +2 more sources
Challenges in the Diagnosis of Cutaneous Mastocytosis
Background: Mastocytosis is characterized by an accumulation of clonal mast cells (MCs) in tissues such as the skin. Skin lesions in mastocytosis may be clinically subtle or heterogeneous, and giving the correct diagnosis can be difficult.
Knut Brockow +9 more
doaj +2 more sources
Phenotypic and genotypic characteristics of mastocytosis according to the age of onset. [PDF]
Adult's mastocytosis is usually associated with persistent systemic involvement and c-kit 816 mutation, while pediatrics disease is mostly limited to the skin and often resolves spontaneously.
Fanny Lanternier +26 more
doaj +9 more sources
Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts
, Cem Akin, Dean D Metcalfe
exaly +2 more sources

