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Requirement for etoposide in the treatment of pregnancy related hemophagocytic lymphohistiocytosis: a multicenter retrospective study

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单位: [1]Department of Hematology, Beijing Friendship Hospital, Capital Medical University, YongAn Road 95th Xicheng District, Beijing 100050, China. [2]Department of Obstetrics and Gynecology, Beijing Friendship Hospital, Capital Medical University, Beijing, China [3]Department of Hematology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China. [4]Department of Obstetrics and Gynecology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China [5]Department of Hematology, Chinese People’s Liberation Army General Hospital, Beijing, China [6]Department of Obstetrics and Gynecology, Chinese People’s Liberation Army General Hospital, Beijing, China [7]Department of Intensive Care Unit, China-Japan Friendship Hospital, Beijing, China [8]Department of Obstetrics and Gynecology, China-Japan Friendship Hospital, Beijing, China.
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关键词: Hemophagocytic lymphohistiocytosis Pregnancy Treatment option Etoposide Corticosteroids

摘要:
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare severe clinical syndrome. HLH manifesting during pregnancy has been paid much attention in recent years. Despite the specificity of pregnancy-related HLH, there has not been any consensus regarding its treatment. According to a previous study, corticosteroid/IVIG is the mainstream therapy; however, the efficacy is controversial. Etoposide is an important agent in the HLH-94 regimen; nevertheless, its use is limited because of possible toxicity to the fetus. Methods: In this study, we summarized 13 cases from 4 medical institutions from April 2011 to April 2018. Treatment regimens and outcomes were observed. Results: The median age was 26 (20-36) years old. The median gestational age was 28 (10-35) weeks. In these 13 patients, 10 were treated with methylprednisolone/IVIG and was effective in only two patients. In 6 patients who used etoposide during their treatment, all achieved remission. The median time from onset of disease to use of etoposide was 36 (17-131) days. Five of these 6 patients were treated with corticosteroids with/without IVIG before etoposide. One patient with pulmonary tuberculosis and one with lymphoma were treated according to etiology and achieved long survival. Conclusion: For treatment of pregnancy-related HLH, particularly for patients who do not respond to corticosteroids/IVIG therapy, etoposide should be used bravely. Nevertheless, suitable dosages and toxic and side-effects require further clinical observation.

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出版当年[2018]版:
大类 | 2 区 医学
小类 | 3 区 遗传学 3 区 医学:研究与实验
最新[2025]版:
大类 | 2 区 医学
小类 | 2 区 遗传学 2 区 医学:研究与实验
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出版当年[2017]版:
Q2 GENETICS & HEREDITY Q2 MEDICINE, RESEARCH & EXPERIMENTAL
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Q2 GENETICS & HEREDITY Q2 MEDICINE, RESEARCH & EXPERIMENTAL

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第一作者单位: [1]Department of Hematology, Beijing Friendship Hospital, Capital Medical University, YongAn Road 95th Xicheng District, Beijing 100050, China.
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