J Pediatr Hematol Oncol. 2026 Jan 20. doi: 10.1097/MPH.0000000000003162. Online ahead of print.
ABSTRACT
OBJECT: Goal of this study was to investigate distribution of 3 aberrant immunophenotypes of T cells in childhood Hemophagocytic lymphohistiocytosis (HLH), and to find their relations with treatment responses and long-time outcomes.
METHODS: Aberrant T cell immunophenotypes presented by patients with HLH at diagnosis during Jan 2018 to Oct 2021 were collected. Distributions of these immunophenotypes among different HLH groups and their relations with first-line therapy responses or lone-time outcomes of patients were studied.
RESULTS: T cell populations with aberrant immunophenotypes were found in 40 patients out of 189 (21.2%). Aberrant immunophenotypic patterns were divided into 3 categories: CD38+HLA-DR+ (N=11, 27.5%), clonal expression of TCRVb (N=17, 42.5%), or down regulation of surface CD5 (N=28, 70.0%). Statistical results showed that T cells from patients with EBV-HLH were prone to present 1 or more of these 3 aberrant immunophenotypes (P<0.001), and that most cases (4/6) with CD4+ T cells with aberrant immunophenotypes were in CAEBV-HLH group. Although plasma levels of IFN-γ were higher in patients with these immunophenotypes (P=0.01), no significant relation was found between these aberrant T cell immunophenotypes and treatment response or long-time outcome. Besides, no hematologic malignancies developed in patients with aberrant T cell immunophenotypes throughout follow up.
CONCLUSION: Patients with HLH frequently show aberrant immunophenotypes of T cells. In most cases, this immunophenotypic patterns have connection to severity, but not outcome of the disease.
PMID:41564346 | DOI:10.1097/MPH.0000000000003162
