title: [The variation and clinical significance of paroxysmal nocturnal hemoglobinuria clone in patients with aplastic anemia before and after immunosuppressive therapy].
abstract: OBJECTIVE
  To evaluate the evolution of paroxysmal nocturnal hemoglobinuria (PNH) clone and its clinical significance before and after immunosuppressive therapy (IST) in patients with aplastic anemia (AA).
  
  
  METHODS
  A total of 186 patients diagnosed as AA were enrolled in this study. Among them, 55 patients were diagnosed as severe AA (SAA) and treated with cyclosporine (CsA) plus anti-thymocyte globulin (ATG), 131 were diagnosed as non SAA (NSAA) and treated with CsA alone. All patients were screened for PNH clone by flow cytometry before treatment and followed up for 18-76 months, with a median time of 22 months.
  
  
  RESULTS
  Positive PNH clones were detected in 10 SAA (18.9%) patients, significantly more than that of NSAA group [9 patients (7.4%), t = 5.041, P = 0.025]. The proportions of PNH clones in SAA group at 6, 12, 24 and > 24 months were 13.38%, 14.88%, 20.00% and 18.85%, respectively, also significantly higher than those of NSAA patients (5.67%, 5.31%, 5.47% and 9.08%, all P values < 0.05). Clinical response rates were comparable in both ATG+CsA or CsA alone groups no matter PNH clone was positive or negative.
  
  
  CONCLUSIONS
  PNH clone are detectable in AA patients either treated with ATG plus CsA or CsA alone, and more significant by ATG plus CsA. Whether PNH clone occurred before or after IST does not affect the therapeutic efficacy.
authors.affiliation: Department of Hematology, the First Affiliated Hospital, Soochow University, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Ying-xin
authors.initials: YX
authors.lastname: Sun
authors.forename: Ming-qing
authors.initials: MQ
authors.lastname: Zhu
authors.forename: Guang-sheng
authors.initials: GS
authors.lastname: He
authors.forename: Xiu-li
authors.initials: XL
authors.lastname: Wang
authors.forename: Bao-zhi
authors.initials: BZ
authors.lastname: Fang
authors.forename: Cong
authors.initials: C
authors.lastname: Lu
authors.forename: Zhen-zhen
authors.initials: ZZ
authors.lastname: Liu
authors.forename: Qian
authors.initials: Q
authors.lastname: Wu
authors.forename: Yong
authors.initials: Y
authors.lastname: Yang
authors.forename: De-pei
authors.initials: DP
authors.lastname: Wu
authors.forename: Ai-ning
authors.initials: AN
authors.lastname: Sun
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000150:complications* / Q000188:drug therapy*; D002648:Child; D002999:Clone Cells; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000209:etiology* / Q000235:genetics; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents