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The effectiveness of the doxorubicin-etoposide-methylprednisolone regimen for adult HLH secondary to rheumatic disease

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Abstract

To investigate the efficacy of the doxorubicin-etoposide-methylprednisolone, DEP) regimen as an effective treatment for adult Hemophagocytic Lymphohistiocytosis secondary to rheumatic disease and analyze prognosis in these patients. Fifty-eight adult patients diagnosed with Hemophagocytic Lymphohistiocytosis secondary to rheumatic disease admitted to Bei**g Friendship Hospital from 1st Jan. 2018 to 31st Dec. 2022 were retrospectively included in this study. Patients were grouped according to previous treatment. Clinical data and laboratory characteristics of patients were retrospectively analyzed. The efficacy was evaluated every 2 weeks after initiating the first course of the DEP regimen and until the last inpatient or 31st Dec. 2023. 26 patients were included in Group A and 32 patients were included in Group B due to the previous treatment. After the first course of the DEP regimen, the overall response rate of all patients was 82.8%, with 13.8% in complete response and 69% in partial response. There was no significant statistical objective response rate between the two groups after the DEP regimen, except at 2-week. Serum ferritin, sCD25, ALT, AST, and DBIL concentrations were significantly lower at 2, 4 and 6-week than pre-treatment (P < 0.05). The overall mortality rate is 20.7% (12/58). Importantly, advanced age, initial level of HB and PLT, and central nervous system (CNS) involvement were independent poor risk factors affecting OS in bivariate analysis. The DEP regimen is effective for adult HLH secondary rheumatic disease with a high overall rate and accepted side effects.

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Funding

This work was supported by the National Natural Science Foundation of China (82370185) and the Key Scientific Project for Capital’s Health Development Research (2020–1-2022).

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Authors and Affiliations

  1. Department of Hematology, Bei**g Friendship Hospital, Capital Medical University, Bei**g, 100050, China

    Dongfei Yin, **gshi Wang & Zhao Wang

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  1. Dongfei Yin
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  2. **gshi Wang
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  3. Zhao Wang
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Contributions

ZW and JSW contributed to the design of the study. DFY conducted the data analysis and wrote the manuscript. All authors approved the final manuscript.

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Correspondence to Zhao Wang.

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The authors declare no competing interests.

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Key messages

1. The DEP regimen is an effective treatment for adult Hemophagocytic Lymphohistiocytosis secondary to rheumatic disease, even patients who exhibited refractory or relapse could achieve remission.

2. The advanced age, baseline level of HB and PLT, and CNS involvement correlated with poor overall survival and progression free survival in MAS.

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Yin, D., Wang, J. & Wang, Z. The effectiveness of the doxorubicin-etoposide-methylprednisolone regimen for adult HLH secondary to rheumatic disease. Ann Hematol (2024). https://doi.org/10.1007/s00277-024-05796-8

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