Clinical study of 6 patients with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis treated with rituximab-containing regimen / 白血病·淋巴瘤

ABSTRACT

Objective To investigate the efficacy of rituximab-containing regimen in Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH). Methods A retrospective analysis involving 6 EBV-HLH patients who had received treatment with rituximab-containing regimen was performed. The patients who were diagnosed with lymphoma or primary HLH subsequently were not included in the analysis. Results All patients were males. The median age was 27.5 years (range 20-61 years). Two patients received rituximab-containing regimen as primary therapy, and got partial remission (PR) within 2 weeks after the first course of rituximab, but relapsed within 4 weeks. Four patients received rituximab-containing regimen as salvage therapy, but none achieved remission. The 6 patients died due to HLH and complications, such as infection and hemorrhage. Laboratory data including white blood cell count, haemoglobin concentration, platelet count ferritin, alanine transaminase, aspartate transaminase,total bilirubin, fibrinogen and EBV-DNA did not show statistical significance (all P>0.05). Conclusion The efficacy of rituximab as a treatment for EBV-HLH is not as good as that in the previous study, and a prospective clinical trial of rituximab-based monotherapy is needed to answer the question.