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Modified DEP regimen as induction therapy for lymphoma-associated hemophagocytic lymphohistiocytosis: a prospective, multicenter study

Purpose Hemophagocytic lymphohistiocytosis (HLH), especially lymphoma-associated HLH (LA-HLH), is a refractory immune disorder with high mortality. There is still no consensus regarding the ideal treatment for LA-HLH. Methods We performed a prospective multicenter study (NCT 04077905) to explore the...

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Published in:Journal of cancer research and clinical oncology 2023-07, Vol.149 (7), p.3033-3041
Main Authors: Pi, Yubo, Wang, Jingshi, Zhou, Hui, Ye, Xiujin, Sun, Xiuhua, Liu, Ligen, Pan, Xueyi, Wang, Zhao
Format: Article
Language:English
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Summary:Purpose Hemophagocytic lymphohistiocytosis (HLH), especially lymphoma-associated HLH (LA-HLH), is a refractory immune disorder with high mortality. There is still no consensus regarding the ideal treatment for LA-HLH. Methods We performed a prospective multicenter study (NCT 04077905) to explore the efficacy of a modified DEP regimen as induction therapy for LA-HLH. Twenty-eight patients from 6 clinical centers in China were enrolled between September 2019 and July 2021. We evaluated the efficacy of the modified DEP induction therapy 4 weeks after the initiation of treatment. Results The results showed that the overall response rate was 89.3% (25/28 patients), whereby 28.6% (8/28 patients) achieved a complete response and 60.7% (17/28 patients) were in partial response. Ferritin and soluble CD25 levels were decreased significantly 4 weeks after the modified DEP induction therapy ( P  = 0.001 and P  = 0.00016, respectively), while platelet count and total bilirubin improved significantly ( P  = 0.004 and P  = 0.001, respectively). The 1-year overall survival rate of all patients was 34.5%, with a median survival of 6.5 months (range 0.5–19 months). Patients with LA-HLH who underwent a stem cell transplantation had a significantly better prognosis than those not achieving complete response 4 weeks after modified DEP induction therapy ( P  = 0.034). Conclusion Our study suggests that the modified DEP regimen is a safe and effective induction therapy for LA-HLH. Timely stem cell transplantation can improve the prognosis of patients with LA-HLH. Trail registry number NCT 04077905. URL: https://clinicaltrials.gov/ct2/show/NCT04077905?id=NCT04077905&draw=2&rank=1 .
ISSN:0171-5216
1432-1335
DOI:10.1007/s00432-022-04157-0