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The hemophagocytic syndrome is a serious clinical-histological entity secondary to different diseases. Collapsing glomerulonephritis is a proliferative podocytopathy that usually has an unfavorable renal prognosis. We present a case in which both entities were associated, which is an infrequent form of hepatosplenic T-cell lymphoma. In addition, we review the role of the markers of podocyte dedifferentiation in this glomerulopathy and its pathophysiology and treatment.
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http://dx.doi.org/10.7705/biomedica.v38i4.3893 | DOI Listing |
Am J Hematol
September 2025
Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.
Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH) is a life-threatening hyperinflammatory syndrome, and hierarchical management based on a prognostic model is important. The endothelial activation and stress index (EASIX) score has demonstrated prognostic utility in recipients of allogeneic stem cell transplantation and chimeric antigen receptor (CAR) T-cell therapy. However, its role in LA-HLH remains unestablished.
View Article and Find Full Text PDFFront Immunol
September 2025
Department of Hematology, Qingdao Women and Children's Hospital, Qingdao, China.
Familial hemophagocytic lymphohistiocytosis type 2 (FHL2), caused by perforin 1 (PRF 1), is a rare and fatal autosomal recessive disorder characterized by a hyperinflammatory syndrome and the accumulation of activated T lymphocytes and histiocytes in the reticuloendothelial system. Autoimmune lymphoproliferative syndrome (ALPS) is an autoimmune disease that typically presents in children with lymphadenopathy, splenomegaly, and cytopenias or lymphomas. We report a case of a 9-year-old boy who was newly diagnosed with FHL, carrying a new type of compound heterozygous mutations (c.
View Article and Find Full Text PDFJ Med Virol
September 2025
Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
Systemic chronic active Epstein-Barr virus disease (sCAEBVD) can primarily involve skeletal muscle to form CAEBV-myopathy (CAEBV-M), which may resemble idiopathic inflammatory myopathies (IIMs). This study reports an 11-patient, 10-year cohort of CAEBV-M to summarize clinicoseropathologic features. CAEBV-M typically affects young adults (median: 29 years), with universal limb swelling, frequent disseminated muscle involvement (73%), and systemic symptoms like fever (82%), splenomegaly (82%), and hemophagocytic lymphohistiocytosis (55%).
View Article and Find Full Text PDFIntern Med
September 2025
Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan.
A 60-year-old woman with rheumatoid arthritis and an impaired consciousness was referred to our hospital. Prior to transfer, the patient underwent intensified immunosuppressive therapy under a diagnosis of rheumatoid vasculitis that had developed in her buttocks. Upon hospitalization, she developed severe multiorgan failure and hemophagocytic syndrome.
View Article and Find Full Text PDFFront Immunol
September 2025
Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, Chongqing, China.
Background: UNC13D deficiency is the most common form of familial hemophagocytic lymphohistiocytosis (FHL) in Asia. Hypogammaglobulinemia is a rare phenotype observed in both patients with FHL3 and sporadic hemophagocytic lymphohistiocytosis (HLH). Our observations suggest that UNC13D deficiency with hypogammaglobulinemia presents a distinct clinical phenotype compared to other HLH patients.
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