Ruxolitinib combined with doxorubicin, etoposide, and dexamethasone for the treatment of the lymphoma-associated hemopha
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ORIGINAL ARTICLE – CLINICAL ONCOLOGY
Ruxolitinib combined with doxorubicin, etoposide, and dexamethasone for the treatment of the lymphoma‑associated hemophagocytic syndrome Lanlan Zhou1 · Yanan Liu1 · Zhenzhen Wen1 · Shimei Yang1 · Mingjie Li1 · Qiuhua Zhu1 · Shiqiu Qiu1 · Yanmin Gao1 · Hong Wang1 · Yuemei Yuan1 · Hanling Zhang1 · Chaolun Chen1 · Wenbin Zeng1 · Zebing Guan1 · Xueyi Pan1 Received: 26 March 2020 / Accepted: 22 June 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
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 38.5 °C and lasting for > 7 days; (2) splenomegaly; (3) hypocytosis (with two or three lineages): hemoglobin (Hb) 6 pedicle rib units by computed tomography (CT), or the spleen was palpated under the rib margin during a physical examination. Spleen shrinkage was defined as CT examination showing spleen shrinkage, or spleen size reduced by 2–3 cm when palpated under the rib margin during a physical examination. During the anti-tumor therapy treatment, the patients were followed every 1–2 months until November 21, 2019. The criteria for treatment response in tumors recommended by the Lugano Classification (2014) were adopted for the treatment response assessment in lymphoma (Cheson et al. 2014). The treatment response was classified as CR, PR, stable disease (SD), and progressive disease (PD). OS was defined as the time from the diagnosis of LAHS to death. The anti-HPS therapy was started on the same day as LAHS diagnosis.
Statistical analysis SPSS 17.0 (SPSS Inc., Chicago, IL) was used for statistical analysis. Continuous data were described as means ± standard deviations, while categorical data were de
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