Abstract
Aim: Epstein-Barr virus (EBV) is the important pathogen of infection after allogeneic hematopoietic stem cell transplantation (allo-HSCT), which is also the common cause of secondary hemophagocytic lymphohistiocytosis (sHLH). Thus, we aimed to identify the clinical manifestations and outcomes of EBV-related sHLH after allo-HSCT.
Methods: We enrolled the patients who experienced sHLH after EBV DNAemia after allo-HSCT from Jan 1st, 2023 to Dec 31st, 2023. Plasma EBV copies were monitored by Q-PCR analysis at least weekly and EBV-DNA copies of lymphocyte subpopulations in peripheral blood were also quantified by Q-PCR.
Results: 11 patients developed sHLH after EBV DNAemia and all of them had post-transplant lymphoproliferative disorders (PTLD) before sHLH. The median time from PTLD to sHLH occurrence was 5 days (range, 2-101) days. The most common manifestation of sHLH was ferritin elevation (100%) and soluble CD25 antigen elevation (100%). Nine (81.8%) patients died after the diagnosis of sHLH, and the median time from the occurrence of sHLH to death was 10 days (range, 3 to 21) days. Temperature ≥ 39.6℃, achieving high-level EBV DNAemia (> 105 copies/ml), and with a high-level EBV in T or NK cells increase the risk of sHLH after EBV-PLTD. The 100-day probability of EBV-related mortality and overall survival after PTLD occurrence was 84.1% versus 8.3 % (P ＜0.0001) and 15.9% versus 91.7% (P ＜0.0001), respectively, for patients with and without sHLH.
Conclusions: In summary, our study firstly reported the clinical manifestations and outcomes of EBV-related sHLH following allo-HSCT in the largest cohort at present.