title: [Treatment outcomes and prognostic analysis of 61 Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis].
abstract: OBJECTIVE
  To investigate the outcomes, survival status, and the prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) patients.
  
  
  METHODS
  A retrospective study was carried out to analyze the clinical data of 61 EBV-HLH cases, from January 2008 to July 2014. Prognostic factors were analyzed through COX model (single factor and multiple factors).
  
  
  RESULTS
  A total of 246 patients with HLH were diagnosed, among which 102 cases were with EBV infection (including 61 EBV-HLH, 36 lymphoma associated HLH, 5 primary HLH. Among the 61 cases, 40 were male and 21 were female, with a ration of 1.9:1. The median age was 28 years (range, 12-78). 1, 3, 6 and 12-month overall survival rates of 61 EBV-HLH were 65.6%, 47.5%, 32.4%, and 25.0%, respectively. The median follow-up time was 3 (0.5-28) months. 12 patients didn't use etoposide within 4 weeks after diagnosis, while HLH-94 protocol was used in 33 patients and HLH-2004 protocol was used in 16 patients. Response rates of theses three groups were 33.3%, 51.5%, and 43.8%, respectively (P=0.401). There was statistically difference between the group without etoposide and the HLH-94/ HLH-2004 group in the overall survival rate (P=0.033). Serum albumin level (P=0.033) and whether EBV could became negative (P=0.010) were independent predictors for EBV-HLH.
  
  
  CONCLUSIONS
  EBV-HLH patients have severe clinical feature and poor prognosis. Early application of immune chemotherapy based on etoposide can improve survival. Serum albumin level and whether EBV can become negative are independent prognostic factors for survival.
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Xiangzong
authors.initials: X
authors.lastname: Zeng
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Na
authors.initials: N
authors.lastname: Wei
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Yi'ni
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Jingshi
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Jia
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Lin
authors.initials: L
authors.lastname: Wu
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Wenqiu
authors.initials: W
authors.lastname: Huang
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Zhuo
authors.initials: Z
authors.lastname: Gao
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Ruijun
authors.initials: R
authors.lastname: Pei
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Jianhang
authors.initials: J
authors.lastname: Chen
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Zhili
authors.initials: Z
authors.lastname: Jin
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing　100050, China.
authors.forename: Zhao
authors.initials: Z
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D020031:Epstein-Barr Virus Infections; D005047:Etoposide; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D005047:Etoposide