Eosinophilic variant of CML (eoCML) is a unique disease with a poor prognosis. Like the hypereosinophilic syndrome (HES), eoCML has no clinically identifiable reason for an increased eosinophil count in the peripheral blood. In contrast to HES, eoCML patients carry a distinct chromosomal abnormality. The bcr/abl fusion gene (Philadelphia chromosome) is the genetic basis of this clonal disease. Recently, eoCML has been separated from HES. Patients with eoCML frequently suffer organ damage including the heart and lungs. This damage is related to the release of eosinophilic granules in the blood, which results in fibrosis of the endothelial lining. We report a case of a peripheral vasculitis complicated by gangrene of the fingers in a patient with eoCML. Despite an almost complete response to CML treatment with Gleevac, combined with prednisone, aspirin and coumadin the patient sustained irreversible damage to the vascular lining of the distal arteries of the upper extremities.
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http://dx.doi.org/10.3109/10428190309178786 | DOI Listing |
Hum Pathol
January 2025
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA 30322, USA. Electronic address:
Introduction: Extraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma defined by NR4A3 gene rearrangements, typically featuring uniform cells with eosinophilic cytoplasm and mild atypia, arranged in cords or clusters within a chondromyxoid stroma. A cellular variant, characterized by increased cellular density and a solid growth pattern, has been recognized.
Methods: We encountered three cases of round cell sarcomas, diagnosed as EMC based on NR4A3 or NR4A2 rearrangements.
Inn Med (Heidelb)
January 2025
Service de gastro-entérologie et d'hepatologie, Centre hospitalier universitaire vaudois (CHUV), Lausanne, Schweiz.
Eosinophilic esophagitis (EoE) was first described in the early 1990s. Initially a rarity, it is now the most common cause of dysphagia for solid foods in young adults. Its prevalence is estimated to be 1:2000.
View Article and Find Full Text PDFJ Allergy Clin Immunol
January 2025
Department of Internal Medicine, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Institute for Medical Immunology, Université Libre de Bruxelles, Brussels, Belgium.
Background: Hypereosinophilic syndrome (HES) is characterized by blood and tissue hypereosinophilia causing organ damage and/or dysfunction. Mepolizumab, an anti-IL-5 antibody, has recently been approved in this indication. In lymphoid variant (L-)HES, eosinophil expansion is driven by IL-5-producing clonal CD3CD4 T cells.
View Article and Find Full Text PDFInt Forum Allergy Rhinol
January 2025
Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.
Background: Dysbiosis of the bacterial and fungal microbiome has been increasingly implicated in the pathogenesis of chronic rhinosinusitis (CRS). This study explores the relationship between microbiome and mycobiome biodiversity and type 2 (T2) versus non-type 2 (NT2) inflammation.
Methods: Mucosal tissues from the ethmoid sinus were collected during endoscopic sinus (CRS) and skull base (controls) surgery between January 2020 and July 2021.
Int J Surg Pathol
January 2025
Department of Pathology, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
Primary intracranial sarcoma, -mutant, included as a new diagnostic entity in the 2021 WHO Classification of Central Nervous System Tumors, is a rare, but aggressive neoplasm generally identified in the supratentorial forebrain. The prognostic implications of these uncommon tumors and optimal treatment strategy remain unclear. A 19-year-old woman was found unresponsive after reporting a severe headache.
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