George H Caughey
George H Caughey
Mast cell granules are packed with proteases, which are released with other mediators by degranulating stimuli. Several of these proteases are targets of potentially therapeutic inhibitors based on hypothesized contributions to diseases, no...
Effect of Gender and Special Considerations for Women in Mastocytosis and Anaphylaxis [0.03%]
性别对 mastocytosis 和过敏性休克的影响及女性患者的特殊注意事项
Sara S Ellingwood,Anna Kovalszki
Sara S Ellingwood
This article reviews the effects of gender on anaphylaxis in general and focuses on mastocytosis-specific issues. Incidence of anaphylaxis is increased in female compared with male patients during the pubertal years through the fifth decade...
Measuring Symptom Severity and Quality of Life in Mastocytosis [0.03%]
测量 mastocytosis患者的症状严重程度和生活质量
Polina Pyatilova,Frank Siebenhaar
Polina Pyatilova
Mastocytosis is a heterogeneous disease with a wide spectrum of signs, symptoms, and concomitant disorders, such as skin lesions, anaphylaxis, osteoporosis, gastrointestinal involvement, and organomegaly. Disease specificity for frequently ...
Tyrosine Kinase Inhibitors in Non-advanced Systemic Mastocytosis [0.03%]
酪氨酸激酶抑制剂在非晚期系统性 mastocytosis 中的应用
Cem Akin
Cem Akin
Systemic mastocytosis is associated with KIT D816V mutation in more than 90% of cases. Patients with non-advanced forms of mastocytosis (indolent systemic mastocytosis, bone marrow mastocytosis, and smoldering systenic mastocytosis) have a ...
Management of Advanced Systemic Mastocytosis and Associated Myeloid Neoplasms [0.03%]
系统性肥大细胞增多症及其相关髓系肿瘤的治疗
Tsewang Tashi,Michael W Deininger
Tsewang Tashi
Advanced systemic mastocytosis (AdvSM) is a heterogeneous group of disorders characterized by neoplastic mast cell-related organ damage and frequently associated with a myeloid neoplasm. The 3 clinical entities that comprise AdvSM are aggre...
Matthew J Hamilton
Matthew J Hamilton
Gastrointestinal symptoms are prevalent in patients with systemic mastocytosis and contribute to morbidity. In indolent disease, the symptoms, which include heartburn, abdominal pain, and diarrhea, are largely due to release of mast cell me...
Matthew P Giannetti,Jennifer Nicoloro-SantaBarbara,Grace Godwin et al.
Matthew P Giannetti et al.
Patients with mastocytosis have an increased risk for mast cell activation events including anaphylaxis when exposed to certain drugs and Hymenoptera venom. Hypotension and cardiovascular collapse without skin or other systemic manifestatio...
Management of Mediator Symptoms, Allergy, and Anaphylaxis in Mastocytosis [0.03%]
mast细胞病的介质症状、过敏和过敏性休克的处理
Theo Gulen
Theo Gulen
Mastocytosis is characterized by expansion and activation of clonally aberrant mast cells (MCs) in one or more organ systems. Inappropriate MC activation is a key finding in both allergy and mastocytosis; therefore, symptoms in both conditi...
Joanna Renke,Ninela Irga-Jaworska,Magdalena Lange
Joanna Renke
To a large extent, the clinical picture of pediatric mastocytosis depends on the age at which it is diagnosed. A neonate with diffuse cutaneous mastocytosis may frequently present in a severe state requiring treatment. Toddlers may require ...
KIT Mutations and Other Genetic Defects in Mastocytosis: Implications for Disease Pathology and Targeted Therapies [0.03%]
肥大细胞增多症中的KIT突变及其他遗传缺陷对其疾病病理及靶向治疗的意义
Yannick Chantran,Peter Valent,Michel Arock
Yannick Chantran
A KIT activating mutation (usually KIT D816V) is detected in neoplastic cells in greater than 90% of indolent patients with systemic mastocytosis (SM). In more advanced variants of SM, additional genetic defects can be found in several myel...