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RASGRP1 Deficiency Associated with Diffuse Mesangial Sclerosis Infantile Nephrotic Syndrome and Epstein-Barr Virus-Induced Hodgkin's Lymphoma.
Pediatr Allergy Immunol Pulmonol
January 2025
Clinical Immunology Unit, Faculty of Medicine and Health Sciences, Department of Paediatrics, Universiti Putra Malaysia, Selangor, Malaysia.
: RAS guanyl-releasing protein 1 (RASGRP1) deficiency is characterized by immune dysregulation and Epstein-Barr virus (EBV)-related lymphoproliferation. Diffuse mesangial sclerosis is one of the infrequent causes of infantile nephrotic syndrome. : Here, we described a 7-year-old girl who was diagnosed with diffuse mesangial sclerosis at 5 months old and subsequently developed chronic bilateral neck swelling at the age of 3 years.
View Article and Find Full Text PDFThe evolving scenario of hcv-related mixed cryoglobulinemia and B-cell lymphoma in the era of direct-acting antivirals.
Expert Rev Anti Infect Ther
January 2025
Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy.
Introduction: Hepatitis C virus (HCV) infection represents a significant global health burden, particularly due to its extrahepatic immune-mediated manifestations, such as mixed cryoglobulinemia, associated vasculitis (CryoVas), and non-Hodgkin B-cell lymphoma (B-NHL), which pose significant challenges. The advent of direct-acting antiviral (DAA) has changed the therapeutic landscape for HCV-related complications.
Areas Covered: This review explores the evolving epidemiology and management of HCV extrahepatic manifestation and lymphoproliferative disorders in the era of DAAs.
Non-Hodgkin Lymphoma of the External Auditory Canal: A Rare Primary Involvement.
Cureus
December 2024
Internal Medicine Department, Unidade Local de Saúde do Nordeste, Bragança, PRT.
The authors describe a rare case of non-Hodgkin lymphoma (NHL) with primary involvement of the external auditory canal (EAC) and subsequent dissemination to the central nervous system, initially manifesting as a benign ear infection. This case highlights the importance of considering differential diagnoses in patients with persistent or worsening symptoms unresponsive to empirical treatment. A 53-year-old man presented with a one-week history of aural fullness, otalgia, and otorrhea in the left ear.
View Article and Find Full Text PDFExtra-Cavitary Primary Effusion Lymphoma in a Patient Co-infected With HIV-1 and HIV-2: A Case Review.
Cureus
December 2024
Infectious Diseases, Hospital Garcia de Orta, Lisbon, PRT.
Extra-cavitary primary effusion lymphoma (PEL), often associated with human herpes virus 8 (HHV8) infection, represents a rare and aggressive form of non-Hodgkin lymphoma, which is predominantly found in individuals with severe immunosuppression. As an acquired immunodeficiency syndrome (AIDS)-associated lymphoma, PEL typically manifests in the context of advanced human immunodeficiency virus (HIV) infection, requiring tailored therapeutic approaches to manage both the lymphoma and underlying immunodeficiency. A 53-year-old male patient from Cape Verde presented with a three-day history of fever, night sweats, right iliac fossa pain, hematochezia, and an unintentional weight loss of five kilograms over the previous two months.
View Article and Find Full Text PDFESR Essentials: imaging of lymphoma-practice recommendations by the European Society of Oncologic Imaging.
Eur Radiol
January 2025
Department of Radiology, NYU Grossman School of Medicine, New York, USA.
Imaging is used for lymphoma detection, Ann Arbor/Lugano staging, and treatment response assessment. [F]FDG PET/CT should be used for most lymphomas, including Hodgkin lymphoma, aggressive/high-grade Non-Hodgkin lymphomas (NHL) such as diffuse large B-cell lymphoma, and many indolent/low-grade NHLs such as follicular lymphoma. Apart from these routinely FDG-avid lymphomas, some indolent NHLs, such as marginal zone lymphoma, are variably FDG-avid; here, [F]FDG PET/CT is an alternative to contrast-enhanced CT at baseline and may be used for treatment response assessment if the lymphoma was FDG-avid at baseline.
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