Hemophagocytic lymphohistiocytosis (HLH) is a rare autosomal recessive disorder of infancy and childhood that is invariably fatal if not treated. We report on the first patient to receive post-natal HSCT for HLH after receiving in utero chemotherapy for disease stabilization.
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http://dx.doi.org/10.1002/pbc.21778 | DOI Listing |
Expert Rev Clin Immunol
December 2024
Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Introduction: Besides cytokine release syndromes (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS), immune effector cell-associated HLH-like syndrome (IEC-HS) is increasingly recognized across CAR-T recipients. This emergent and fatal syndrome is difficult to separate from other disorders during the early phase, and urgently requires more integrated diagnostic and therapeutic frameworks.
Areas Covered: Existing literature has pointed out the potential role of unbridled proliferation of cytotoxic T lymphocytes, lymphopenia of natural killing cells, and hypercytokinemia in triggering the IEC-HS.
J Immunother
December 2024
Department of Hematology, Peking University First Hospital, Beijing, China.
Secondary hemophagocytic lymphohistiocytosis (HLH) syndrome, a fatal disorder characterized by NK/T-cell deficiency, cytokine storm, and organ damage, is rare in chronic lymphocytic leukemia (CLL). Ibrutinib, the first generation of irreversible Bruton's tyrosine kinase inhibitor, has been the first-line therapy for CLL. As an off-target effect, it can also block IL-2 inducible T-cell kinase (ITK), which is essential in maintaining normal NK and T-cell functions.
View Article and Find Full Text PDFJ Infect
December 2024
Guangzhou Medical Research Institute of Infectious Diseases, Infectious Disease Center, Guangzhou Eighth People's Hospital, Guangzhou Medical University, Guangzhou, Guangdong 510440, China. Electronic address:
Transpl Immunol
December 2024
National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Department of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China. Electronic address:
Hemophagocytic lymphohistiocytosis (HLH) is a severe disorder with poor clinical outcomes. Use of emapalumab, an IFN-γ inhibitor, enables primary HLH control in over 85 % of affected children. However, data on emapalumab use for Epstein-Barr virus-associated HLH (EBV-HLH) are limited.
View Article and Find Full Text PDFJ Allergy Clin Immunol
December 2024
Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Mass. Electronic address:
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