Báo cáo y học: "Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options"

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. | Molderings et al. Journal of Hematology Oncology 2011 4 10 http content 4 1 10 JOURNAL OF HEMATOLOGY ONCOLOGY REVIEW Open Access Mast cell activation disease a concise practical guide for diagnostic workup and therapeutic options Gerhard J Molderings1 Stefan Brettner2 Jurgen Homann3 Lawrence B Afrin4 Abstract Mast cell activation disease comprises disorders characterized by accumulation of genetically altered mast cells and or abnormal release of these cells mediators affecting functions in potentially every organ system often without causing abnormalities in routine laboratory or radiologic testing. In most cases of mast cell activation disease diagnosis is possible by relatively non-invasive investigation. Effective therapy often consists simply of antihistamines and mast cell membrane-stabilising compounds supplemented with medications targeted at specific symptoms and complications. Mast cell activation disease is now appreciated to likely be considerably prevalent and thus should be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity or patients in whom a definitively diagnosed major illness does not well account for the entirety of the patient s presentation. Introduction The term mast cell activation disease MCAD denotes a collection of disorders characterized by 1 accumulation of pathological mast cells in potentially any or all organs and tissues and or 2 aberrant release of variable subsets of mast cell mediators. A classification has been proposed which differentiates several types and subclasses of MCAD Table 1 . The traditionally recognized subclass termed systemic mastocytosis SM includes disorders characterized by certain pathological immunohistochemical and mutational findings the WHO criteria Table 2 1 2 which are divided into several subtypes Table 1 . On the other hand mast cell activation syndrome MCAS presents a complex clinical picture of multiple mast cell mediator-induced .

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