title: [Clinical characteristics of 192 adult hemophagocytic lymphohistiocytosis].
abstract: OBJECTIVE
  To analyze the clinical manifestations, laboratory data, therapy, and prognosis in patients with hemophagocytic lymphohistiocytosis (HLH).
  
  
  METHODS
  A retrospective study was carried out in 192 adult patients with HLH between 2003 and 2013.
  
  
  RESULTS
  Of the 192 cases, 70 cases were secondary to cancer and 64 cases secondary to infection. According to HLH-2004 criteria, the coincidence rate of indices were: fever (98.96%), high level of serum ferritin (94.27%), increased level of soluble interleukin- 2 receptor(sCD25) (94.79%), decreased or absent activity of NK cells (94.27%), cytopenias (80.73%), splenomegaly (80.21%), emophagocytosis in bone marrow, spleen or lymph nodes (74.48%), hypofibrinogenemia (50.52%), hypertriglyceridemia (37.50%). In addition, 94.27% of patients were presented with liver dysfunction, 96.35% with infections, and 75.52% with coagulopathy. Incidences of central nervous system symptoms and rash were 19.27% and 20.31%, respectively. Among cancer, infection and rheumatic group, there were statistically differences on white blood cells (WBC), platelet (PLT), sCD25, alanine aminotransferase, aspartate aminotransferase, total bilirubin and globulin(GLO) (P<0.05). The differences of WBC, PLT, albumin (ALB), GLO, brain natriuretic peptide, creatinine, urea nitrogen between survival group and death group had statistical significance.
  
  
  CONCLUSIONS
  The secondary HLH occurs from various underlined diseases. Cancer, especially T- cell lymphoma, is the main cause, Secondly, it is EB virus infection. The diagnostic sensitive indicators are Persistent fever, higher level of serum ferritin, low or absent NK-cell activity, and increased sCD25 were the most valuable parameters for diagnosis. Cytopenias were not common in early phase of HLH secondary to rheumatic diseases. WBC, PLT, ALB, GLO could be used as the preliminary parameters for diagnosis. Cardiac insufficiency, renal insufficiency and coagulation dysfunction play important roles in prognosis.
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: Yini
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: Shuo
authors.initials: S
authors.lastname: Li
authors.affiliation: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
authors.forename: Ran
authors.initials: R
authors.lastname: Tang
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: Jianhang
authors.initials: J
authors.lastname: Chen
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: Zhao
authors.initials: Z
authors.lastname: Wang
mesh_terms: D000328:Adult; D001853:Bone Marrow; D005334:Fever; D006801:Humans; D007694:Killer Cells, Natural; D051359:Lymphohistiocytosis, Hemophagocytic; D011379:Prognosis; D012189:Retrospective Studies