Publications by authors named "J I Henter"
Etoposide has revolutionized the treatment of primary as well as secondary hemophagocytic lymphohistiocytosis (HLH), and it is, together with corticosteroids, the most widely used therapy for HLH. In the early 1980s, long-term survival in primary HLH was <5% but with the etoposide-/dexamethasone-based protocols HLH-94 and HLH-2004, in combination with stem cell transplantation, 5-year survival increased dramatically to around 60% in primary HLH, and based on analyses from the HLH-2004 study, there is likely room for further improvement. Biologically, etoposide administration results in potent selective deletion of activated T cells as well as efficient suppression of inflammatory cytokine production.
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- The study analyzed the current diagnostic criteria for familial hemophagocytic lymphohistiocytosis (FHL) and aimed to improve them through a case-control approach involving 366 children with genetic FHL or Griscelli syndrome.
- It compared the existing HLH-2004 criteria's effectiveness with a new optimal model based on 17 variables, finding similar diagnostic thresholds with high accuracy rates (99.1% overall).
- The researchers concluded that while the HLH-2004 criteria are valid, additional cellular and genetic assays are beneficial for confirming diagnoses, particularly in differentiating FHL from severe infections or systemic-onset juvenile idiopathic arthritis.
Primary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder associated with autosomal recessive variants in genes required for perforin-mediated lymphocyte cytotoxicity. A rapid diagnosis is crucial for successful treatment. Although defective cytotoxic T lymphocyte (CTL) function causes pathogenesis, quantification of natural killer (NK)-cell exocytosis triggered by K562 target cells currently represents a standard diagnostic procedure for primary HLH.
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- Stromal cells are crucial for maintaining the balance of epithelial and immune cells and are significant in the development of inflammatory bowel disease (IBD).
- The research investigates the stromal response to inflammation in pediatric IBD, identifying specific inflammatory reactions in different parts of the colon and intestinal layers.
- Findings show that certain fibroblasts and monocytes/macrophages interact closely in the intestine, with fibroblasts promoting the conversion of monocytes into a specific type of macrophage that resembles those found in young IBD patients, indicating the stroma's role in guiding macrophage development.