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Prognostic impact of expression of CD2, CD25, and/or CD30 in/on mast cells in systemic mastocytosis: a registry study of the European Competence Network on Mastocytosis.
Leukemia
January 2025
Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
Expression of CD2, CD25 and/or CD30 in extracutaneous mast cells (MC) is a minor diagnostic criterion for systemic mastocytosis (SM) in the classification of the World Health Organization and International Consensus Classification. So far, it remains unknown whether expression of these antigens on MC is of prognostic significance in SM. We performed a retrospective multi-center study of patients with SM using the data set of the registry of the European Competence Network on Mastocytosis, including 5034 patients with various MC disorders.
View Article and Find Full Text PDFHow I diagnose and treat systemic mastocytosis with an associated hematologic neoplasm.
Blood
January 2025
Guy's & St Thomas' NHS Foundation Trust, London, United Kingdom.
Over the last decade significant advances have been made by honing in on the diagnostic evaluation and the significance of molecular profiles in patients with systemic mastocytosis (SM), non-advanced and advanced.This is reflected in the 2022 iterations of the World Health Organization Edition 5 and International Consensus Criteria classifications.The impact of targeted KIT inhibitor therapies on patients treated within global trials has demonstrated significant improvements in the prognosis and overall survival for patients, leading to a change the treatment paradigm.
View Article and Find Full Text PDFRegistries in allergy: Structure, target groups, and key findings of allergy-focused registries in Germany.
Allergol Select
December 2024
Division of Allergy and Immunology, Department of Dermatology, Venerology and Allergology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
In allergology, clinical registries fill knowledge gaps of epidemiology, mechanisms of allergic diseases, and real-world treatment outcomes. Considering the continuous rise of allergic diseases worldwide, registries become increasingly important for the optimization and harmonization of patient care. In the current review, we present four ongoing allergy-focused registries initiated in Germany.
View Article and Find Full Text PDFEfficacy of valaciclovir in preventing herpes zoster in patients receiving anifrolumab.
RMD Open
January 2025
Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière, Centre de Référence des maladies auto-immunes et auto-inflammatoires systémiques rares de l'adulte d'Ile-de-France, Centre et Martinique, Service de Médecine Interne 2, Institut E3M, Paris, France, paris, France.
Lifetime Disability-adjusted Life Year assessment of indolent systemic mastocytosis.
J Allergy Clin Immunol Pract
December 2024
CEREMAST, Dermatology Department, University Hospital, Toulouse, France.
Background: Indolent Systemic Mastocytosis (ISM) is a rare disease associated with numerous and diverse symptoms that significantly impact patients' overall health, psychological, emotional, and professional well-being, ultimately affecting their quality of life.
Objective: We aim to estimate the Disability-adjusted Life Year (DALY) of ISM to assess the burden for patients and society.
Methods: We used prospective and retrospective data on symptoms and quality of life from an ISM population recruited at the French expert center CEREMAST, to estimate Disability Weight allowing DALY calculation.
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