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Pleural Rosai-Dorfman disease complicated with renal clear cell carcinoma: a case report and literature review.
Front Oncol
January 2025
Department of Radiology, Daping Hospital, Army Medical University, Chongqing, China.
Background: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant disorder characterized by excessive proliferation of histiocytes, the cause of which remains unknown. Although the lymph nodes are the most commonly affected site, some patients may present with extranodal involvement, particularly in the skin, nasal cavity, eyes, and bones. In this report, we aim to present a unique case of RDD with pleural involvement in a 61-year-old patient.
View Article and Find Full Text PDFA hidden enemy: Understanding the hemophagocytic syndrome in children under five years of age in a high-complexity institution in southwestern Colombia.
Biomedica
December 2024
Facultad de Ciencias de la Salud, Universidad ICESI, Cali, Colombia, Departamento de Pediatría, Alergología e Inmunología Pediátrica, Fundación Valle del Lili, Cali, Colombia.
Introduction. Hemophagocytic syndrome is an under-recognized condition with high mortality in the pediatric population. It is characterized by excessive activation of immune cells and cytokine release, leading to persistent inflammation.
View Article and Find Full Text PDFBackground: Hemophagocytic lymphohistiocytosis (HLH) is a rare complication of multiple myeloma (MM), with limited data available on its incidence, clinical presentation, and treatment. The underlying mechanisms linking MM and HLH remain unclear, including the potential role of MM treatment agents in triggering HLH.
Methods: This case report presents a patient with MM who developed HLH while on lenalidomide maintenance therapy.
Griscelli syndrome: Erdheim-Chester disease-like local presentation progressing to accelerated phase.
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 PDFHemophagocytic Lymphohistiocytosis in a Critically Ill Patient: A Case Report of a Potentially Fatal Entity.
HCA Healthc J Med
December 2024
Oakland University William Beaumont School of Medicine, Rochester Hills, MI.
Background: Hemophagocytic lymphohistiocytosis (HLH) is a non-neoplastic proliferation and macrophage activation that induces cytokine-mediated bone marrow suppression and features of intense phagocytosis in the bone marrow and liver, leading to multi-organ dysfunction and ultimate failure. The diagnosis of HLH in an intensive care setting is challenging, and it is associated with high morbidity and mortality. HLH-94 is the standard protocol for treatment, consisting of dexamethasone and chemotherapy like etoposide.
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