title: Central nervous system involvement in mantle cell lymphoma: clinical features, prognostic factors and outcomes from the European Mantle Cell Lymphoma Network.
abstract: BACKGROUND
  Central nervous system (CNS) involvement in mantle cell lymphoma (MCL) is uncommon, and the manifestations and natural history are not well described.
  
  
  METHODS
  We present the data on 57 patients with MCL who developed CNS involvement, from a database of 1396 consecutively treated patients at 14 institutions.
  
  
  RESULTS
  The crude incidence of CNS involvement was 4.1%, with 0.9% having CNS involvement at diagnosis. Blastoid histology, B-symptoms, elevated lactate dehydrogenase, Eastern Cooperative Group performance status ≥2 and a high Mantle Cell Lymphoma International Prognostic Index score were enriched in the cohort with CNS involvement, and the presence of ≥1 of these features defined a high-risk subset (an actuarial risk of CNS involvement 15% at 5 years) in a single-institution subset. The median time to CNS relapse was 15.2 months, and the median survival from time of CNS diagnosis was 3.7 months. The white blood cell count at diagnosis <10.9 × 10⁹/l, treatment of CNS involvement with high-dose anti-metabolites, consolidation with stem cell transplant and achievement of complete response were all associated with improved survival.
  
  
  CONCLUSIONS
  In MCL, CNS involvement is uncommon, although some features may predict risk. Once manifest outlook is poor; however, some patients who receive intensive therapy survive longer than 12 months.
authors.affiliation: Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Australia.
authors.forename: C Y
authors.initials: CY
authors.lastname: Cheah
authors.forename: A
authors.initials: A
authors.lastname: George
authors.forename: E
authors.initials: E
authors.lastname: Giné
authors.forename: A
authors.initials: A
authors.lastname: Chiappella
authors.forename: H C
authors.initials: HC
authors.lastname: Kluin-Nelemans
authors.forename: W
authors.initials: W
authors.lastname: Jurczak
authors.forename: K
authors.initials: K
authors.lastname: Krawczyk
authors.forename: H
authors.initials: H
authors.lastname: Mocikova
authors.forename: P
authors.initials: P
authors.lastname: Klener
authors.forename: D
authors.initials: D
authors.lastname: Salek
authors.forename: J
authors.initials: J
authors.lastname: Walewski
authors.forename: M
authors.initials: M
authors.lastname: Szymczyk
authors.forename: L
authors.initials: L
authors.lastname: Smolej
authors.forename: R L
authors.initials: RL
authors.lastname: Auer
authors.forename: D S
authors.initials: DS
authors.lastname: Ritchie
authors.forename: L
authors.initials: L
authors.lastname: Arcaini
authors.forename: M E
authors.initials: ME
authors.lastname: Williams
authors.forename: M
authors.initials: M
authors.lastname: Dreyling
authors.forename: J F
authors.initials: JF
authors.lastname: Seymour
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D002490:Central Nervous System / Q000473:pathology*; D016543:Central Nervous System Neoplasms / Q000188:drug therapy / Q000517:prevention & control / Q000556:secondary*; D005060:Europe; D005260:Female; D006801:Humans; D007958:Leukocyte Count; D020522:Lymphoma, Mantle-Cell / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012306:Risk; D013534:Survival; D016896:Treatment Outcome
chemical_list: D000964:Antimetabolites, Antineoplastic
keywords: central nervous system; mantle cell lymphoma; prognosis