Background: Primary hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by immune dysregulation. Hematopoietic stem cell transplantation (HSCT) represents the only option for long-term cure for primary HLH. However, only around 25% of patients have a fully HLA-matched donor.
View Article and Find Full Text PDFMediterr J Hematol Infect Dis
May 2024
The aim of this study was to investigate the prognostic factors of haploid hematopoietic stem cell transplantation in the treatment of X-linked lymphoproliferative syndrome. Seven children with X-linked lymphoproliferative syndrome diagnosed by XIAP gene analysis were enrolled. The conditioning regimens were tolerated in all seven patients, and the median time of neutrophil engraftment was 10 days (8-13 days), and that of platelet engraftment was 21 days (14-24 days).
View Article and Find Full Text PDFAllogeneic hematopoietic stem cell transplantation (HSCT) currently stands as the sole remedy for individuals afflicted with hemophagocytic lymphohistiocytosis (HLH). In this study, we retrospectively evaluated how pediatric patients with relapsed or refractory (R/R) HLH responded to our institution's cocktail conditioning regimen. The disease was diagnosed according to criteria applicable to patients with familial/genetic, relapsing, or severe/persistent HLH.
View Article and Find Full Text PDFIndian J Hematol Blood Transfus
July 2023
This study aims to assess the efficacy of second allogeneic hemopoietic stem cell transplantation (allo-HSCT) for treating hemophagocytic syndrome with first engraftment failure. Among a total of 35 patients who underwent allo-HSCT between June 2015 and July 2021 for HLH, 10 patients who underwent a second HSCT following graft rejection were retrospectively analyzed. Various factors, such as the treatment course and outcome, the remission status, donor selection, and the conditioning regimen of patients before second allo-HSCT, were scrutinized for transplant-related complications and transplant-related mortality, as well as transplant outcomes.
View Article and Find Full Text PDFAllogeneic hematopoietic stem cell transplantation (allo-HSCT) was considered as an only therapeutic strategy for chronic active Epstein-Barr virus (CAEBV) infection with few exceptions, while efficacy of various allo-HSCT conditioning regimens for CAEBV has not been fully investigated yet. This study aimed to compare the effectiveness of cocktail conditioning regimen (CCR)-allo-HSCT with reduced-intensity conditioning regimen (RICR)-allo-HSCT for pediatric patients with CAEBV. Data of a total of 54 children with CAEBV from July 2015 to December 2020 were retrospectively analyzed.
View Article and Find Full Text PDFPediatr Transplant
February 2019
Objective: To investigate the efficacy of ACBT for acquired SAA in children.
Methods: Four patients including three girls and one boy were diagnosed with acquired SAA. Their ages were 5, 3, 1, and 3 years, respectively.
Reactive oxygen species are believed to play a role in the development of several diseases including vascular diseases and the aging process. It is reported that increased reactive oxygen species were implicated as an important mechanism that contributes to endothelial dysfunction. So, human umbilical cord vein endothelial cells were used to study the antioxidative effect of L-ascorbic acid and its derivative.
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