Publications by authors named "Mouel E"
Article Synopsis
- Advanced systemic mastocytosis (AdvSM) is a complex condition linked to poor outcomes, and while midostaurin is the first approved treatment, its long-lasting effectiveness is limited.
- Various prognostic scoring systems like MARS, IPSM, and GPSM have been developed to assess patients' outcomes, but it's essential to tailor these scores to specific AdvSM subtypes for better accuracy.
- A study of patients treated with midostaurin revealed that MARS and AdvSM subtype significantly predict overall survival, identifying five distinct patient subgroups with varying median survival times, highlighting the need for personalized management strategies.
Systemic mastocytosis (SM) corresponds to a rare and heterogeneous spectrum of diseases characterized by the accumulation of atypical mast cells (MCs). Advanced mastocytosis (Adv-SM) is associated with poor survival; in contrast, patients with non-advanced SM (non-Adv-SM) usually have a normal life expectancy but may experience poor quality of life. Despite recent therapeutic progress including tyrosine kinase inhibitors, new treatment options are needed for refractory and/or intolerant patients with both severely symptomatic and Adv-SM.
View Article and Find Full Text PDFBackground And Aims: Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology of SM has been marginally described and accurate histological classification is critical, given the consequences of aggressive SM diagnosis. We aimed to describe the histological features associated with liver SM using updated tools.
View Article and Find Full Text PDFBackground: Mastocytosis and monoclonal mast cell (MC) activation syndrome (MMAS) are heterogeneous conditions characterized by the accumulation of atypical MCs. Despite the recurrent involvement of KIT mutations, the pathophysiologic origin of mastocytosis and MMAS is unclear. Although hereditary α-tryptasemia (HαT, related to TPSAB1 gene duplication) is abnormally frequent in these diseases, it is not known whether the association is coincidental or causal.
View Article and Find Full Text PDFEffective Anti-SARS-CoV-2 Immune Response in Patients With Clonal Mast Cell Disorders.
J Allergy Clin Immunol Pract
May 2022
Background: Mast cells are key players in innate immunity and the T2 adaptive immune response. The latter counterbalances the T1 response, which is critical for antiviral immunity. Clonal mast cell activation disorders (cMCADs, such as mastocytosis and clonal mast cell activation syndrome) are characterized by abnormal mast cell accumulation and/or activation.
View Article and Find Full Text PDFArticle Synopsis
- This study evaluates the safety and effectiveness of TNF-α antagonists and tocilizumab in treating Takayasu arteritis (TAK) in 209 patients, primarily young women.
- Results show that both treatment options achieved a high rate of complete response (66% for TNF-α and 70% for tocilizumab) after 6 months, although older age was positively correlated with response while certain baseline symptoms were negatively associated.
- The incidence of treatment relapses and adverse effects was similar for both therapies, indicating that they are equally effective for managing refractory TAK over a median follow-up of 36 months.
Introduction: Adult-onset Still's Disease is a rare multisystemic inflammatory disease characterized by fever, maculo-papular erythematous rash and arthralgia. Adult-onset Still's disease is a diagnosis of exclusion.
Case Report: We report the case of a 33 years old man, hospitalized for fever, arthralgia and throat manifestations, leading to Adult-onset Stills's Disease diagnosis.