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| http://dx.doi.org/10.1007/s00277-023-05598-4 | DOI Listing |
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Breaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.
Proc (Bayl Univ Med Cent)
July 2024
Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated.
View Article and Find Full Text PDFTocilizumab and immune signatures for targeted management of cytokine release syndrome in immune checkpoint therapy.
Ann Oncol
December 2024
Centre Hospitalier Universitaire Vaudois (CHUV), University of Lausanne, Department of Medicine, Immunology and Allergy Service, Rue du Bugnon 46, CH-1011 Lausanne, Switzerland. Electronic address:
Background: This study aimed to identify specific biomarkers in oncology patients experiencing immune-related cytokine release syndrome (irCRS)-like symptoms during immune checkpoint inhibitor (ICI) therapy, including severe cases like hemophagocytic lymphohistiocytosis (irHLH), and to distinguish these from sepsis. A secondary objective was to retrospectively analyze the efficacy of tocilizumab (TCZ) in treating corticosteroid (CS)-refractory high-grade irCRS.
Patients And Methods: A cohort of 35 patients presenting with irCRS-like symptoms was studied, including 9 with irHLH-like manifestations and 8 with sepsis.
Uncommon biphasic CAR-T expansion induces hemophagocytic lymphohistiocytosis-like syndrome and fatal multiple infections following BCMA CAR-T cell therapy: a case report.
J Immunother Cancer
November 2024
State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China
B-cell maturation antigen(BCMA)-directed chimeric antigen receptor (CAR)-T-cell therapy has significantly improved the treatment of relapsed or refractory multiple myeloma (MM). Nevertheless, the uncommon phenomenon of biphasic CAR-T cell expansion in vivo and its related severe toxicities have not been methodically described and studied. Herein, we report a case of patients with MM who experienced two CAR-T cell expansion peaks and subsequently developed multiple severe toxicities following BCMA CAR-T cell infusion.
View Article and Find Full Text PDFCBL syndrome presenting with severe EBV infection and panuveitis masquerade.
Eur J Ophthalmol
November 2024
Department of Ophthalmology, IHU FOReSIGHT, Hôpital Universitaire Pitié-Salpêtrière, Sorbonne Université, Paris, France.
Article Synopsis
- This case report discusses a 35-month-old boy with CBL syndrome who experienced life-threatening Epstein-Barr virus (EBV) infections and rod-cone dystrophy (RCD) that appeared similar to severe panuveitis.
- Genetic testing indicated a pathogenic variant related to CBL syndrome, leading to complications including encephalitis and severe eye inflammation.
- The report emphasizes the importance of considering RCD in CBL syndrome patients with unexplained eye inflammation and suggests using carbonic anhydrase inhibitors as a treatment for cystoid macular edema.
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