Clinical features and outcomes of patients with hemophagocytic lymphohistiocytosis at onset of systemic autoinflammatory disorder and compare with Epstein-Barr virus (EBV)-related hemophagocytic lymphohistiocytosis.
机构：[a]Beijing Key Laboratory of Pediatric Hematology Oncology, Capital Medical University, National Center for Children’s Health, Beijing,China.[b]National Key Discipline of Pediatrics (Capital Medical University), Capital Medical University, National Center for Children’s Health, Beijing,China.[c]Key Laboratory of Major Diseases inChildren, Ministry of Education, Capital Medical University, National Center for Children’s Health, Beijing,China.[d]Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing,China.医技科室职能科室临床流行病与循证医学中心血液中心首都医科大学附属北京儿童医院
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. In clinical practice, we have observed that some HLH patients who have features of systemic autoinflammatory diseases (SAIDs) exhibit unique clinical manifestations and outcomes different from other HLH patients.We analyzed data from 25 HLH patients who were considered to have SAIDs; data were collected from patients of our center between January 1, 2015 and September 1, 2018.The median age of the patients was 1.75 years. In the early phase, all patients had a fever and 92% of patients had a rash; 96% of patients had high white blood cell count (WBC), C-reaction protein, and erythrocyte sedimentation rate. With progression, the above laboratory results decreased gradually. During the HLH period, we compared SAIDs-related HLH and Epstein-Barr virus (EBV)-related HLH and found that rash was more common (92%, P < .001) and splenomegaly was less common (64%, P = .023) in SAIDs-related HLH. Further, WBC, ferritin, and Interleukin-6 levels in SAIDs-related HLH patients were higher than those in EBV-related HLH patients. In contrast, hemoglobin, triglyceride, sCD25, Interleukin-10, and interferon-γ levels in SAIDs-related HLH patients were lower compared with those in EBV-related HLH patients. SAIDs-related HLH patients received a modified HLH-2004 protocol at our center. Most patients had a good prognosis.We provide a summary of the unique clinical and laboratory features, treatment protocols, and outcomes of SAIDs patients with HLH at onset. The findings indicate that these patients had a better response to corticosteroids and cyclosporin compared with EBV-related HLH patients.
基金：
National Science and Technology Key Projects [2017ZX09304029004]; Beijing Municipal Science and Technology CommissionBeijing Municipal Science & Technology Commission [Z171100001017050]; National Natural Science Foundation of ChinaNational Natural Science Foundation of China [81700186, 81800189]; Scientific Research Common Program of Beijing Municipal Commission of EducationBeijing Municipal Commission of Education [KM201710025019]; Talent Training Project-Fostering Fund of National Natural Science Foundation of Beijing Children's Hospital, Capital Medical University [GPY201713]; Special Fund of The Pediatric Medical Coordinated Development Center of Beijing Municipal Administration [XTZD20180202]; Guangdong Province Key Laboratory of Popular High Performance Computers of Shenzhen University [SZU-GDPHPCL2017]
第一作者机构：[a]Beijing Key Laboratory of Pediatric Hematology Oncology, Capital Medical University, National Center for Children’s Health, Beijing,China.[b]National Key Discipline of Pediatrics (Capital Medical University), Capital Medical University, National Center for Children’s Health, Beijing,China.[c]Key Laboratory of Major Diseases inChildren, Ministry of Education, Capital Medical University, National Center for Children’s Health, Beijing,China.[d]Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing,China.
通讯作者：
通讯机构：[a]Beijing Key Laboratory of Pediatric Hematology Oncology, Capital Medical University, National Center for Children’s Health, Beijing,China.[b]National Key Discipline of Pediatrics (Capital Medical University), Capital Medical University, National Center for Children’s Health, Beijing,China.[c]Key Laboratory of Major Diseases inChildren, Ministry of Education, Capital Medical University, National Center for Children’s Health, Beijing,China.[d]Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing,China.[*1]Beijing Children’s Hospital, Nanlishi Road No. 56, Xicheng District, Beijing 100045, China
推荐引用方式(GB/T 7714)：
Yunze Zhao,Zhigang Li,Li Zhang,et al.Clinical features and outcomes of patients with hemophagocytic lymphohistiocytosis at onset of systemic autoinflammatory disorder and compare with Epstein-Barr virus (EBV)-related hemophagocytic lymphohistiocytosis.[J].Medicine.2020,99(1):e18503.doi:10.1097/MD.0000000000018503.
APA：
Yunze Zhao,Zhigang Li,Li Zhang,Hongyun Lian,Honghao Ma...&Rui Zhang.(2020).Clinical features and outcomes of patients with hemophagocytic lymphohistiocytosis at onset of systemic autoinflammatory disorder and compare with Epstein-Barr virus (EBV)-related hemophagocytic lymphohistiocytosis..Medicine,99,(1)
MLA：
Yunze Zhao,et al."Clinical features and outcomes of patients with hemophagocytic lymphohistiocytosis at onset of systemic autoinflammatory disorder and compare with Epstein-Barr virus (EBV)-related hemophagocytic lymphohistiocytosis.".Medicine 99..1(2020):e18503
