title: [Clinical study of DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis].
abstract: OBJECTIVE
  To investigate the efficacy of liposomal doxorubicin together with etoposide and high dose methylprednisolone (DEP) as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis (HLH).
  
  
  METHODS
  Total 41 patients with refractory HLH were enrolled in this study. The efficacy of treatment with DEP regimen after 2 and 4 weeks were evaluated according to the United States Midwest Cooperative HLH Group.
  
  
  RESULTS
  Of 41 refractory HLH patients, 28 were males and 13 females. The median age was 31(18-62) years old. The overall response rate (ORR) was 78.1%(32/41), including 12 patients (29.3%) achieved complete remission (CR) and 20 (48.8%) achieved partial remission (PR). The underlying disease of HLH were identified in 33 patients, including 1 case of primary HLH (CR), 20 cases of lymphoma associated HLH and 12 cases of EBV associated HLH. There were still 8 cases with unknown underlying disease. The 9 patients who had no response to DEP died within 2 to 4 weeks after salvage therapy. Twenty of the 32 patients who achieved PR or CR survived to undergo subsequent chemotherapy, allogenic hematopoietic stem cell transplantation (allo-HSCT) or splenectomy.
  
  
  CONCLUSIONS
  The single-arm study suggested that DEP regimen appeared to be an effective salvage protocol for adult patients with refractory HLH.
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: Wenqiu
authors.initials: W
authors.lastname: Huang
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: Xiangzong
authors.initials: X
authors.lastname: Zeng
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: Jingshi
authors.initials: J
authors.lastname: Wang
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: Li
authors.initials: L
authors.lastname: Fu
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; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D002945:Cisplatin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D016879:Salvage Therapy; D055815:Young Adult
chemical_list: D005047:Etoposide; D002945:Cisplatin; D011241:Prednisone