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Paraneoplastic pemphigus and Castleman's disease: a case report and a revision of the literature.
Ital J Pediatr
March 2023
Pediatric Unit, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
Background: In literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman's disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis.
Case Presentation: We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms.
Castleman's Disease with Pulmonary Artery Aneurysm: A Rare Presentation of Behçet's Disease.
Cureus
April 2020
Rheumatology, King Fahad Specialist Hospital, Dammam, SAU.
Diagnosing Behcet's disease (BD) in a patient already diagnosed with Castelman's disease (CD) is rare. There are only a few cases reported in the literature and all of them were diagnosed as BD prior to the patient experiencing symptoms and signs of CD. We present a patient who was initially diagnosed as having CD.
View Article and Find Full Text PDFDiversity of XMEN Disease: Description of 2 Novel Variants and Analysis of the Lymphocyte Phenotype.
J Clin Immunol
February 2020
Department of Laboratory Immunology, PathWest QEII Medical Centre, Perth, WA, Australia.
Variants in MAGT1 have been identified as the cause of an immune deficiency termed X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection and neoplasia (XMEN) disease. Here, we describe 2 cases of XMEN disease due to novel mutations in MAGT1, one of whom presented with classical features of XMEN disease and another who presented with a novel phenotype including probable CNS vasculitis, HHV-8 negative multicentric Castelman disease and severe molluscum contagiosum, thus highlighting the clinical diversity that may be seen in this condition. Peripheral blood immunophenotyping of these 2 patients, together with an additional 4 XMEN patients, revealed reduced NKG2D expression, impaired CD28 expression on CD8 T cells, CD4 T cell lymphopenia, an inverted CD4:CD8 ratio and decreased memory B cells.
View Article and Find Full Text PDF[Castleman's Disease - A Case Series of a Rare Lymphoproliferative Disease].
Laryngorhinootologie
August 2017
Klinik für Hals-Nasen-Ohrenheilkunde, Universitätsklinikum Ulm.
Castleman's disease is a very rare and potentially severe lymphoproliferative disorder. First sign may be cervical lymphadenitis, requiring sufficient support in diagnosis and therapy by an ENT specialist. Based on a case series the current manuscript gives an overview of the symptoms, the course of disease and the therapy options.
View Article and Find Full Text PDFOcular adnexal lymphoproliferative disorders in an ophthalmic referral center in Saudi Arabia.
Saudi J Ophthalmol
July 2013
King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.
Purpose: To study the pattern of ocular adnexal lymphoproliferative disorders (OALD) in an ophthalmic referral center in Saudi Arabia and to review their, histopathological characteristics with clinical correlation.
Methods: Retrospective chart review of 40 cases of patients who underwent incisional biopsy with the suspected diagnosis of periocular and/or adnexal lymphoid lesions over the period: 2000-2012 at the King Khaled Eye Specialist Hospital (KKESH), Riyadh, Saudi Arabia. The routine histopathologic slides are reviewed by a single pathologist to identify cases of Benign Reactive Lymphoid Hyperplasia (RLH), Atypical Lymphoid Hyperplasia and probable lymphoma.
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