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IgG4-related disease for the hematologist.
Hematology Am Soc Hematol Educ Program
December 2024
Division of Hematology, University of British Columbia, Vancouver, British Columbia, Canada; and Division of Hematology, Dalhousie University, Halifax, Nova Scotia, Canada.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis.
View Article and Find Full Text PDFOrbital granular cell tumor involving the superior rectus muscle: a case report.
Front Oncol
November 2024
Department of Orbital Surgery, Chongqing Aier Eye Hospital, Chongqing, China.
Objective: The aim of this case report is to assess the clinicopathological characteristics and differential diagnosis of orbital granular cell tumor (GCT).
Methods: Clinical and imaging data of a rare case of orbital GCT involving the superior rectus muscle were collected. Its clinical characteristics, imaging, and histopathological features were observed.
Unusual Presentation of Synovial Lipomatosis Mimicking As Septic Arthritis of Knee: A Case Report.
Cureus
April 2024
Orthopaedics and Traumatology, Chettinad Hospital and Research Institute, Chennai, IND.
Synovial lipomatosis or lipoma arborescens is a very uncommon pseudo-tumorous lesion of the synovium which more commonly affects the knee joint. The most probable cause of this pathological lesion is degenerative articular disorders of the joint and improper fat accumulation. It is characterized by presence of villous proliferation of the synovium and replacement of the sub-synovial tissue by mature adipocytes which is infiltrated by dense chronic inflammatory cells like lymphocytes, plasma cells and eosinophils.
View Article and Find Full Text PDFIgG4-related disease: Case report and 6-year follow-up of an elusive diagnosis mimicking malignancy.
Clin Case Rep
May 2024
Family Health Unit of Barrinha, Esmoriz Local Health Unit of the Aveiro Region Aveiro Portugal.
Key Clinical Message: IgG4-related disease is a rare and emerging pathology, characterized by the appearance of pseudotumors. Due to the ability to mimic other pathologies, it is essential to consider it as a differential diagnosis in multisystemic processes. The diagnosis is challenging, requiring a multidisciplinary approach, to minimize the associated morbidity and mortality.
View Article and Find Full Text PDFPhotodynamic therapy for cutaneous inflammatory pseudotumour: A case report.
Photodiagnosis Photodyn Ther
February 2024
Department of Dermatology, First Medical Center of Chinese PLA General Hospital, Beijing, 100853, PR China. Electronic address:
Inflammatory pseudotumour (IP) is a rare proliferative disease characterized by a dense infiltrate of plasma cells, lymphocytes, eosinophils and neutrophils in the fibrous stroma. It primarily affects the lungs of pediatric patients or young adults. Cutaneous IP is an extremely rare condition, with limited documentation in the English literature.
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