Abstract
Purpose
Case reports suggest that ruxolitinib-containing treatment could increase the clinical response rate of patients with hemophagocytic syndrome (HPS). This study aimed to explore the effect of ruxolitinib-containing treatment for patients with lymphoma-associated hemophagocytic syndrome (LAHS).
Methods
This was a retrospective study of patients with LAHS hospitalized at the First Affiliated Hospital of Guangdong Pharmaceutical University between October 2017 and September 2019. Patients were treated with HLH-94 (etoposide and dexamethasone) or R-DED regimen (ruxolitinib, doxorubicin, etoposide, and dexamethasone). The clinical characteristics, treatment responses, and overall survival (OS) were compared. The patients were divided into the HLH-94 group (n = 34) and the R-DED group (n = 36).
Results
Compared with HLH-94, R-DED might effectively improve the clinical manifestations, including fever and splenomegaly in patients with LAHS, and control the systemic cytokine storm. The response rate at 2 weeks was 54.8% in the HLH-94 group, which was lower than in the R-DED group (83.3%) (p = 0.011). The OS was significantly prolonged in the R-DED group compared with the HLH-94 group (median, 5 vs. 1.5 months, p = 0.003). The multivariable analysis showed that lower IL-10 levels [hazard ratio (HR)] = 1.000, [95% confidence interval (CI)] 1.000–1.000, p = 0.012), R-DED regimen (HR = 0.196, 95% CI 0.084–0.457, p < 0.001), and non-NK/T-cell lymphoma (HR = 0.254, 95% CI 0.102–0.628, p = 0.003) were associated with better OS. The prognosis of patients with LAHS was generally poor.
Conclusion
Ruxolitinib can be combined with chemotherapy in HPS. It is feasible, with no early signals of increased toxicity.
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Availability of data and materials
The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.
Code availability
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Funding
This study was supported by the Natural Science Foundation of Guangdong Province (2017A030313664), the Science and Technology Project of Yuexiu District of Guangzhou (2017-W S-008), the Medical Science and Technology Research Funding of Guangdong Province (A2019518), and the Science and Technology Planning Project of Guangzhou (202002030253).
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LZ and YL conceived and coordinated the study, designed, performed, and analyzed the experiments, wrote the paper. ZW, SY, ML, QZ, SQ, YG, HW, YY, HZ, CC, WZ, ZG, and XP carried out the data collection, data analysis, and revised the paper. All authors reviewed the results and approved the final version of the manuscript.
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Zhou, L., Liu, Y., Wen, Z. et al. Ruxolitinib combined with doxorubicin, etoposide, and dexamethasone for the treatment of the lymphoma-associated hemophagocytic syndrome. J Cancer Res Clin Oncol 146, 3063–3074 (2020). https://doi.org/10.1007/s00432-020-03301-y
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DOI: https://doi.org/10.1007/s00432-020-03301-y