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Successful treatment with Omalizumab of a child affected by Systemic Mastocytosis: clinical and biological implications
BackgroundPediatric Mastocytosis is a rare and heterogeneous disease, characterized by accumulation of mast cells in the skin (Cutaneous...
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Sugammadex in systemic mastocytosis
Perioperative management in patients suffering from systemic mastocytosis is challenging. Most recommendations regarding anesthetic management in...
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Allogeneic haematopoietic cell transplantation for advanced systemic mastocytosis: Best practice recommendations on behalf of the EBMT Practice Harmonisation and Guidelines Committee
Systemic Mastocytosis (SM) is a multifaceted clinically heterogeneous disease. Advanced SM (AdvSM) comprises three entities: aggressive SM (ASM),...
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Systemic mastocytosis mimicking blastic plasmacytoid dendritic cell neoplasm: a case report
BackgroundSystemic mastocytosis (SM), a rare myeloid neoplasm, is defined as a clonal and neoplastic proliferation of mast cells in at least one...
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Psychometric evaluation of the Indolent Systemic Mastocytosis Symptom Assessment Form (ISM-SAF©) and determination of a threshold score for moderate symptoms
BackgroundThe Indolent Systemic Mastocytosis Symptom Assessment Form (ISM-SAF) (©Blueprint Medicines Corporation), a 12-item daily diary that...
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Perioperative management of mastocytosis
PurposePatients with mastocytosis have an increased risk of anaphylaxis during surgical procedures with general anesthesia. Therefore, we reviewed...
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Response and resistance to cladribine in patients with advanced systemic mastocytosis: a registry-based analysis
We sought to evaluate the efficacy of the purine analogue cladribine in 79 patients with advanced systemic mastocytosis (AdvSM) using data from the...
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The international consensus classification of mastocytosis and related entities
Mastocytosis is a neoplasm characterized by a clonal proliferation of mast cells, which accumulate in one or multiple organs, associated with an...
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Avapritinib in the Treatment of Systemic Mastocytosis: an Update
Purpose of ReviewPatients with systemic mastocytosis, a dangerous and rare myeloid neoplasm, have long had few therapies available to them and,...
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Efficacy of avapritinib versus best available therapy in the treatment of advanced systemic mastocytosis
Advanced systemic mastocytosis (AdvSM) is a rare myeloid neoplasm associated with poor overall survival (OS). This study (NCT04695431) compared...
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Cognitive Impairment and Depression in Mastocytosis: A Synthesis of the Literature
Purpose of ReviewSymptoms of depression and cognitive dysfunction are commonly reported in mastocytosis. The aims of this review paper are to...
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Allogeneic Hematopoietic Cell Transplantation in Advanced Systemic Mastocytosis: A retrospective analysis of the DRST and GREM registries
We identified 71 patients with AdvSM (aggressive SM [ASM], SM with an associated hematologic neoplasm [SM-AHN, e.g., acute myeloid leukemia, SM-AML],...
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Triggers of Anaphylaxis in Mastocytosis Patients: Evidence of the Current Drug-Avoidance Recommendation
Mastocytosis is associated with a high risk of anaphylaxis, in part due to drug hypersensitivity reactions (DHR). Drugs associated with mast cell...
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Health-related quality of life and health literacy in patients with systemic mastocytosis and mast cell activation syndrome
BackgroundSystemic mastocytosis is a rare genetic disease characterized by aberrant proliferation and/or activation of mast cells, resulting in...
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Development of symptom-focused outcome measures for advanced and indolent systemic mastocytosis: the AdvSM-SAF and ISM-SAF©
BackgroundAdvanced systemic mastocytosis (AdvSM), indolent systemic mastocytosis (ISM), and smoldering systemic mastocytosis (SSM) are rare diseases...
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Psychometric evaluation of the Indolent Systemic Mastocytosis Symptom Assessment Form (ISM-SAF) in a phase 2 clinical study
BackgroundIndolent systemic mastocytosis (ISM) is a rare, clonal mast cell neoplasm characterized by severe, unpredictable symptoms. The Indolent...
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Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis
In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic...
