Chronic active Epstein-Barr virus (EBV) infection is a rare chronic mononucleosis syndrome involving clonally proliferating EBV-infected T-/NK-cells. EBV DNA was quantified in nonpleocytotic cerebrospinal fluid (CSF) of 9 patients. Three patients with neurologic and/or neuroimaging abnormalities showed high CSF copy numbers. In 1 patient, CSF copy number exceeded the peripheral blood value. CSF EBV-load may predict the central nervous system involvement of EBVT-/NK-cells.
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http://dx.doi.org/10.1097/INF.0b013e318178d21e | DOI Listing |
Turk J Pediatr
November 2024
Division of Pediatric Immunology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Türkiye.
Background: Griscelli syndrome (GS) is a rare genetic disorder characterized by oculocutaneous albinism and variable immune dysfunction. Among three distinct types of GS, occurring due to different genetic mutations; GS type 1 presents with neurological manifestations, hemophagocytic lymphohistiocytosis (HLH) generally develops in GS type 2, and GS type 3 primarily exhibits oculocutaneous albinism. HLH, a life-threatening condition with excessive immune activation, may occur secondary to various triggers, including infections, and develop in different tissues, as well as in the testis, similar to Erdheim-Chester disease.
View Article and Find Full Text PDFChin Clin Oncol
December 2024
Colorectal Cancer Center, Sichuan University West China Hospital, Chengdu, China; Department of Medical Oncology, Cancer Center, Sichuan University West China Hospital, Chengdu, China.
Background: Epstein-Barr virus-associated gastric cancer (EBVaGC) is characterized by higher lymphocytic infiltration, which predicts sensitivity to immunotherapy. However, there are few studies investigating the mechanisms of acquired resistance to programmed cell death protein 1 (PD-1) blockade and its subsequent treatment strategies for EBVaGC.
Case Description: We describe the case of a patient with EBVaGC who was initially treated with first-line chemotherapy plus Sintilimab, a fully humanized anti-PD-1 monoclonal antibody, resulting in a near-complete response.
Turk Arch Pediatr
January 2025
Division of Allergy and Immunology, Department of Pediatrics, Marmara University Faculty of Medicine, İstanbul, Türkiye.
Objective: Prolidase deficiency is a metabolic and immunological disorder that is inherited in an autosomal recessive manner. In prolidase deficiency, a broad spectrum of differences is observed in patients, ranging from asymptomatic to multisystem involvement. There is scarce information in the literature on the atypical features and immunophenotypes of this disease.
View Article and Find Full Text PDFAm J Surg Pathol
January 2025
Department of Pathology, Health Research Institute-Fundación Jímenez Diaz University Hospital. Universidad Autónoma de Madrid (IIS-FJD, UAM).
Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive malignancy that frequently presents with extranodal involvement. Cutaneous tropism is clinically and histopathologically variable, which may pose a diagnostic challenge. We conducted a retrospective analysis of 40 samples of 20 cases of cutaneous AITL, focusing on the clinicopathologic and molecular correlations between skin and lymph node (LN) samples.
View Article and Find Full Text PDFAm J Cancer Res
December 2024
Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital, Linkou, Chang Gung University Taoyuan 33305, Taiwan.
Nasopharyngeal carcinoma (NPC) is an Epstein-Barr virus (EBV)-associated cancer, and immune checkpoint inhibitors (ICIs) have shown efficacy in its treatment. The combination of chemotherapy and ICIs represents a new trend in the standard care for metastatic NPC. In this study, we aim to clarify the immune cell profile and related prognostic factors in the ICI-based treatment of metastatic NPC.
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