title: Central nervous system relapse in adults with acute lymphoblastic leukemia.
abstract: BACKGROUND
  Recurrence of acute lymphoblastic leukemia (ALL) in the central nervous system (CNS) in adults is considered a poor prognostic feature. Few reports have analyzed this issue. The objective of this study was to analyze the experience with CNS recurrence in adult patients with ALL.
  
  
  METHODS
  Between December 1982 and April 2000, the records of 527 consecutive, newly diagnosed patients with ALL who were treated at the M. D. Anderson Cancer Center were reviewed. Patients were treated with one of two published regimens: vincristine, doxorubicin, and dexamethasone (VAD; n = 237 patients) or hyperfractionated cyclophosphamide plus VAD (hyper-CVAD) (n = 290 patients). Among 439 patients (83%) who achieved complete remission (CR), 32 patients (7%) had a CNS recurrence. All patients had received CNS prophylaxis tailored to the risk of CNS recurrence with systemic chemotherapy with or without intrathecal (IT) chemotherapy; none received cranial radiotherapy for prophylaxis. The records of these patients were reviewed.
  
  
  RESULTS
  The median age at the time of diagnosis was 33 years. The median CR duration prior to CNS recurrence was 36 weeks (range, 2-185 weeks). Three groups were identified: 1) patients with an isolated CNS recurrence (n = 17 patients), 2) patients with CNS recurrences after bone marrow (BM) recurrence (n = 8 patients), and 3) patients with simultaneous CNS and BM recurrences (n = 7 patients). IT chemotherapy was effective in achieving a CNS CR in 30 patients (94%), but 10 patients (31%) had a second CNS recurrence. The median survival from CNS recurrence was 6 months; 28% of patients were alive at 1 year, and 6% of patients were alive at 4 years.
  
  
  CONCLUSIONS
  Adults with ALL and CNS recurrences have a poor prognosis despite effective IT chemotherapy. Future studies should investigate better approaches in the treatment of these patients to improve their long-term survival. Effective CNS prophylaxis remains the single best approach for treating patients with CNS leukemia.
authors.affiliation: Department of Leukemia, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA.
authors.forename: Usha R
authors.initials: UR
authors.lastname: Surapaneni
authors.forename: Jorge E
authors.initials: JE
authors.lastname: Cortes
authors.forename: Deborah
authors.initials: D
authors.lastname: Thomas
authors.forename: Susan
authors.initials: S
authors.lastname: O'Brien
authors.forename: Francis J
authors.initials: FJ
authors.lastname: Giles
authors.forename: Charles
authors.initials: C
authors.lastname: Koller
authors.forename: Stefan
authors.initials: S
authors.lastname: Faderl
authors.forename: Hagop
authors.initials: H
authors.lastname: Kantarjian
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016543:Central Nervous System Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000556:secondary*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control / Q000532:radiotherapy; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis