title: [Children's NK/T Cell Lymphoma-Associated Hemophagocytic Syndrome: Clinical Analysis of 6 Cases].
abstract: OBJECTIVE
  To study the clinical features, treatment and prognosis of patients with NK/T cell lymphoma-associated hemophagocytic syndrome(NK/T-LAHPS).
  
  
  METHODS
  Retrospective analysis was used to explore the clinical data of 6 children with NK/T-LAHPS who were admitted in Department of Pediatric Hematology of Fujian Medical University Union Hospital from July 2012 to June 2016. The 6 patients included 4 boys and 2 girls, with a median age of 4 years(range 1.75 to 11). In 4 patients the hemophagocytic syndrome(HPS) occurred as the main primary manifestations of underlying lymphoma, in the other 2 patients HPS occurred during lymphoma progression. The clinical manifestations included persistent fever(6/6), hepatomegaly(6/6), splenomegaly(6/6) and pancytopenia(6/6). Laboratory data indicated that the level of ferritin(2179-15000 ng/ml) , LDH(608-3899 IU/L) and EBV-DNA(>105copies/ml ) was elevated obviously. The other common clinical features of NK/T-LAHPS were hypoproteinemia(6/6), hepatic dysfunction(5/6), hypofibrinogenimia(5/6), hypertriglyceridemia(3) and hemophagocytosis in bone marrow(5/6).
  
  
  RESULTS
  After being treated according to the HLH-2004 protocol combined with supported therapy for 1 or 2 weeks, all the patients achieved a clinical response, and the laboratory indicators of HPS were improved. The combined chemotherapy of SMILE was given to 4 patients timely, among them 2 patients achieved complete remission(CR) and long term survival, 1 patient achieved partial remission(PR) and died of relapse after drug withdrawal and 1 patient died of aggravated lymphoma. The other 2 patients did not receive chemotherapy in time, HPS relapsed quickly, because of the progression of lymphoma, and all died of severe hepatic dysfunction and coagulopathy.
  
  
  CONCLUSIONS
  The NK/T-LAHPS is an invariably fatal disease with poor prognosis, and typically occurrs at the advanced stage or the terminal phase of the disease. HLH-2004- based protocol in combination with comprehensive therapy is hopeful for the patients with NK/T-LAHPS, which may delay the disease progression and provide opportunities for the treatment of primary disease. Once the laboratory indicators of HPS are improved, it is important to treat lymphoma timely with the combined chemotherapy of SMILE, which is significant for improving the prognosis.
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Yong-Zhi
authors.initials: YZ
authors.lastname: Zheng
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Hao
authors.initials: H
authors.lastname: Zheng
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Shao-Hua
authors.initials: SH
authors.lastname: LE
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Xue-Ling
authors.initials: XL
authors.lastname: Hua
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Zai-Sheng
authors.initials: ZS
authors.lastname: Chen
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Ling
authors.initials: L
authors.lastname: Zheng
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Mei
authors.initials: M
authors.lastname: Li
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Yi-Qiao
authors.initials: YQ
authors.lastname: Chen
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Qin-Li
authors.initials: QL
authors.lastname: Gao
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China.
authors.forename: Jing-Hui
authors.initials: JH
authors.lastname: Yang
authors.affiliation: Department of Hematology, Fujian Medical University Union Hospital, Fujian Institute of Hematology, Fuzhou 350001, Fujian Province, China. E-mail:drjiandahu@163.com.
authors.forename: Jian-Da
authors.initials: JD
authors.lastname: Hu
mesh_terms: D001853:Bone Marrow; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic; D016411:Lymphoma, T-Cell, Peripheral; D008297:Male; D009364:Neoplasm Recurrence, Local; D010198:Pancytopenia; D011379:Prognosis; D012189:Retrospective Studies