Rituximab-containing immuno-chemotherapy regimens are effective for the elimination of EBV for EBV-HLH with only and mainly B lymphocytes of EBV infection

Int Immunopharmacol. 2021 Jul:96:107606. doi: 10.1016/j.intimp.2021.107606. Epub 2021 Apr 5.

Abstract

Patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) have a poor prognosis. This study investigated the efficacy of rituximab-containing immuno-chemotherapy regimens for EBV-HLH. In this study, 15 patients were treated with rituximab-containing regimens. The treatment efficacy and adverse events were evaluated. In 10 patients, EBV DNA became negative after the first course of treatment. The lymphocyte types infected by EBV in the 10 patients were only infected with B cells and mainly infected with B cells. In the other 5 patients, the EBV DNA of peripheral blood mononuclear cells (PBMC) before and after treatment with the regimens had no statistical difference (P = 0.111). In addition, in these 5 patients, EBV mainly infected T and NK cells. Among the 5 patients without a significant decline in EBV DNA of PBMC, 2 patients received allogeneic hematopoietic stem cell transplantation and turned negative for EBV DNA. This study suggests that rituximab-containing regimens are effective therapy for EBV-HLH with only and mainly B lymphocytes infected by EBV, especially for eliminating EBV.

Keywords: Epstein-Barr virus; Hemophagocytic lymphohistiocytosis; Rituximab.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • B-Lymphocytes / drug effects*
  • B-Lymphocytes / virology
  • Child
  • Child, Preschool
  • Drug Therapy, Combination
  • Epstein-Barr Virus Infections / blood
  • Epstein-Barr Virus Infections / complications
  • Epstein-Barr Virus Infections / drug therapy*
  • Epstein-Barr Virus Infections / immunology*
  • Female
  • Herpesvirus 4, Human / drug effects
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / immunology
  • Humans
  • Immunologic Factors / adverse effects
  • Immunologic Factors / pharmacology*
  • Immunologic Factors / therapeutic use
  • Infant
  • Leukocytes, Mononuclear / drug effects
  • Leukocytes, Mononuclear / virology
  • Lymphohistiocytosis, Hemophagocytic / blood
  • Lymphohistiocytosis, Hemophagocytic / complications
  • Lymphohistiocytosis, Hemophagocytic / drug therapy*
  • Lymphohistiocytosis, Hemophagocytic / immunology*
  • Male
  • Middle Aged
  • Rituximab / adverse effects
  • Rituximab / pharmacology*
  • Rituximab / therapeutic use
  • Survival Rate
  • Treatment Outcome
  • Young Adult

Substances

  • Immunologic Factors
  • Rituximab