单位:[1]Department of Hematology, Beijing Friendship Hospital, Capital Medical University, YongAn Road 95th Xicheng District, Beijing 100050, China.临床科室血液科血液科首都医科大学附属北京友谊医院[2]Department of Obstetrics and Gynecology, Beijing Friendship Hospital, Capital Medical University, Beijing, China临床科室妇产科妇产科首都医科大学附属北京友谊医院[3]Department of Hematology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.北京朝阳医院[4]Department of Obstetrics and Gynecology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China北京朝阳医院[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.
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.
基金:
Beijing Natural Science FoundationBeijing Natural Science Foundation [7181003]; Beijing Municipal Administration of Hospitals' Ascent Plan [DFL20180101]
语种:
外文
被引次数:
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PubmedID:
中科院(CAS)分区:
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
JCR分区:
出版当年[2017]版:
Q2GENETICS & HEREDITYQ2MEDICINE, RESEARCH & EXPERIMENTAL
最新[2024]版:
Q2GENETICS & HEREDITYQ2MEDICINE, RESEARCH & EXPERIMENTAL
第一作者单位:[1]Department of Hematology, Beijing Friendship Hospital, Capital Medical University, YongAn Road 95th Xicheng District, Beijing 100050, China.
通讯作者:
推荐引用方式(GB/T 7714):
Song Yue,Wang Zhao,Hao Zengping,et al.Requirement for etoposide in the treatment of pregnancy related hemophagocytic lymphohistiocytosis: a multicenter retrospective study[J].ORPHANET JOURNAL of RARE DISEASES.2019,14:doi:10.1186/s13023-019-1033-5.
APA:
Song, Yue,Wang, Zhao,Hao, Zengping,Li, Lihong,Lu, Junli...&Chen, Bo.(2019).Requirement for etoposide in the treatment of pregnancy related hemophagocytic lymphohistiocytosis: a multicenter retrospective study.ORPHANET JOURNAL of RARE DISEASES,14,
MLA:
Song, Yue,et al."Requirement for etoposide in the treatment of pregnancy related hemophagocytic lymphohistiocytosis: a multicenter retrospective study".ORPHANET JOURNAL of RARE DISEASES 14.(2019)