A female patient with the history and clinical signs suggesting cutaneous and systemic mastocytosis is reported. The diagnosis of cutaneous mastocytosis was confirmed by the Darier's sign as well as by histologic analysis of the skin. An X-ray film of the bones showed an infiltration of the mast cells into the skeletal system what was also cytologically and histologically proven. The patient was treated only symptomatically because of the benign course of the disease. A current literature data on mastocytosis syndrome are discussed.
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Blood Adv
January 2025
University of Michigan, Ann Arbor, Michigan, United States.
Systemic mastocytosis (SM) is a rare disease and has had significant discoveries in its biology, prognostication, and management in the past two decades. The latest update of the WHO classification and the new ICC classification are current standards in the diagnosis and prognostication of SM. In clinical practice, SM can be divided into two main categories: non-advanced SM (nonAdvSM) and advanced SM (AdvSM).
View Article and Find Full Text PDFLeukemia
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 PDFBlood
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 PDFDiagnostics (Basel)
January 2025
Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, 80-214 Gdańsk, Poland.
We present an interesting image of eruptive syringoma confirmed by histopathological assessment in a 37-year-old male who was consulted due to numerous brownish small macules and papules resembling maculopapular cutaneous mastocytosis (MPCM). We show difficulties in diagnosing ES, given its rare occurrence and resemblance to other dermatological disorders. Moreover, we discuss the role of dermoscopy and reflectance confocal microscopy in the differential diagnosis of syringoma.
View Article and Find Full Text PDFClin Exp Allergy
January 2025
Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
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