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Pre-diagnostic trajectory of pediatric hemophagocytic lymphohistiocytosis: observations from hematological and hepatic parameters.
Ann Hematol
December 2024
Pediatric Intensive Care Unit, Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan Children's Hospital), Changsha, China.
Understanding the early features and characteristics of hemophagocytic lymphohistiocytosis (HLH) is essential for identifying high-risk individuals and also providing valuable pathological insights. This study aims to investigate the characteristics and trends of blood and hepatic parameters before an HLH diagnosis was established. Longitudinal hematological and hepatic test results from pediatric patients with HLH and an age- and sex-matched control group were analyzed.
View Article and Find Full Text PDFLate-Onset Hemophagocytic Lymphohistiocytosis in a Lung Transplant Patient: A Case of T-Cell Post-Transplant Lymphoproliferative Disorder.
Am J Case Rep
October 2024
Department of Pneumology, CHU UCLouvain Namur, Yvoir, Belgium.
Article Synopsis
- Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome that can occur in individuals with genetic predispositions or in immunocompromised patients, though it’s particularly rare in solid organ transplant recipients, such as lung transplant patients.
- This case study presents an unusual incident of HLH developing 11 years after lung transplantation in a 67-year-old patient, with symptoms including pancytopenia, fever, and splenomegaly, and showed signs of post-transplant lymphoproliferative disorder (PTLD).
- Despite treatment with etoposide, corticosteroids, and rituximab, the patient ultimately died from complications, highlighting the need for
Accelerated phase development in a late-onset adolescent Chediak-Higashi syndrome patient caused by compound novel LYST mutations in the setting of SARS-CoV-2 infection.
Blood Cells Mol Dis
November 2024
Department of Laboratory Medicine, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China. Electronic address:
Chediak-Higashi syndrome (CHS) is a rare autosomal recessive genetic disorder characterized by severe immunodeficiency, albinism and coagulation deficiency. Mostly diagnosed in early childhood, this devastating condition is associated with lysosomal abnormalities attributed to the absence or impaired function of lysosomal trafficking regulator caused by mutations in the CHS1/LYST gene. In current study, we report a case of late-onset CHS caused by two novel compound heterozygous CHS1/LYST mutations: c.
View Article and Find Full Text PDFTiming of Toxicities and Non-Relapse Mortality Following CAR T Therapy in Myeloma.
Transplant Cell Ther
September 2024
Division of Hematologic Malignancies and Cellular Therapeutics, University of Kansas Cancer Center, Westwood, Kansas; U.S. Myeloma Innovations Research Collaborative, Kansas City, Kansas.
BCMA-directed chimeric antigen receptor T-cell (CAR T) therapies, including idecabtagene vicleucel (ide-cel) and ciltacabtagene autoleucel (cilta-cel), have transformed the treatment landscape for relapsed-refractory multiple myeloma (RRMM), offering remarkable efficacy with hallmark toxicity risks of cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). The FDA mandates a 4-week monitoring period at the treatment center as part of a Risk Evaluation and Mitigation Strategy (REMS) to monitor and manage these toxicities, which, while prudent, may add unnecessary challenges related to access and socioeconomic disparities. We sought to assess CRS and ICANS onset and duration, as well as causes of non-relapse mortality (NRM) in real-world BCMA CAR T recipients in order to better inform future changes to the monitoring guidelines for CAR T recipients.
View Article and Find Full Text PDFLate-onset Familial Hemophagocytic Lymphohistiocytosis in a survivor of Hodgkin's Lymphoma.
Leuk Res Rep
November 2023
Department of Oncology, The Aga Khan University Hospital, Karachi, Pakistan.
Hemophagocytic Lymphohistiocytosis is an inflammatory condition which results in over activation of the immune system. It could be either sporadic or familial. The familial subtype is linked with various genetic mutations and is commonly a disease of the young.
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