title: Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China.
abstract: BACKGROUND
  Hemophagocytic lymphohistiocytosis (HLH) is a relatively rare but life-threatening disease with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality.
  
  
  METHODS
  To improve the recognition as well as understanding of this disorder, we analyzed clinical characteristics and prognostic factors from 85 adult patients diagnosed with HLH in our hospital from April 2005 to June 2014.
  
  
  RESULTS
  Patients with HLH displayed variable clinical markers across a wide spectrum. These included fever and hyperferritinemia (100%), elevated lactate dehydrogenase (LDH) (98.8%), two or three cytopenia (92.2%), splenomegaly (72.9%), hypofibrinogenemia (69.4%), hypertriglyceridemia (64.7%), hemophagocytosis (51.7%), and hepatomegaly (24.7%). Patients with active Epstien-Barr Virus (EBV) infection had a median overall survival (OS) of 65 days. Those displaying malignancy had very poor survival (median OS: 40 days). However, patients in rheumatic and non-EBV infection groups had relatively superior prognosis (not reached). Univariate analysis showed that Fibrinogen (Fbg) <1.5 g/L, platelet number (PLT) <40 × 10(9)/L and LDH ≥1000 U/L were factors that negatively affected survival (P = 0.004, 0.000, 0.002). Multivariate analysis showed that PLT <40 × 10(9)/L was the independent adverse factor (HR = 0.350, 95% CI: 0.145-0.844, P = 0.019).
  
  
  CONCLUSIONS
  HLH had very complex clinical manifestations and high death rate. Patients with active EBV infection, malignancy, Fbg <1.5 g/L, PLT <40 × 10(9)/L and LDH ≥1000 U/L had high risk of death as well as inferior survival, and these patients require systemic targeted treatments as early as possible.
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanchang University, NanChang, China. yx021021@sina.com.
authors.forename: Fei
authors.initials: F
authors.lastname: Li
authors.affiliation: Department of Urology, Tulane University Health Science Center, New Orleans, LA, USA. yijun.yang@tulane.edu.
authors.forename: Yijun
authors.initials: Y
authors.lastname: Yang
authors.affiliation: Tumor Center, The First Hospital of Jilin University, Changchun, China. lxcjfy@163.com.
authors.forename: Fengyan
authors.initials: F
authors.lastname: Jin
authors.affiliation: Department of Pathology, University of Iowa Carver College of Medicine, Iowa City, USA. casey-dehoedt@uiowa.edu.
authors.forename: Casey
authors.initials: C
authors.lastname: Dehoedt
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanchang University, NanChang, China. raojia2004126@sina.com.
authors.forename: Jia
authors.initials: J
authors.lastname: Rao
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanchang University, NanChang, China. wenxin_yl@163.com.
authors.forename: Yulan
authors.initials: Y
authors.lastname: Zhou
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanchang University, NanChang, China. 13970998685@163.com.
authors.forename: Pu
authors.initials: P
authors.lastname: Li
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanchang University, NanChang, China. yangganping@medmail.com.cn.
authors.forename: Ganping
authors.initials: G
authors.lastname: Yang
authors.affiliation: State Key Laboratory of Natural Medicines, School of Life Science and Technology, China Pharmaceutical University, Nanjing, China. minwang@cpu.edu.cn.
authors.forename: Min
authors.initials: M
authors.lastname: Wang
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanchang University, NanChang, China. yx021021@sina.com.
authors.forename: Rongyan
authors.initials: R
authors.lastname: Zhang
authors.affiliation: State Key Laboratory of Natural Medicines, School of Life Science and Technology, China Pharmaceutical University, Nanjing, China. yangye876@sina.com.
authors.forename: Ye
authors.initials: Y
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000900:Anti-Bacterial Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D002681:China; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use; D005047:Etoposide / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D007722:Health Knowledge, Attitudes, Practice; D004854:Herpesvirus 4, Human; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000139:chemically induced / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology*; D008223:Lymphoma / Q000150:complications / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000900:Anti-Bacterial Agents; D000970:Antineoplastic Agents; D005938:Glucocorticoids; D005047:Etoposide; D016572:Cyclosporine