title: Central nervous system involvement in mantle cell lymphoma.
abstract: BACKGROUND
  Extranodal involvement, including central nervous system (CNS), is a frequent event in patients with mantle cell lymphoma (MCL). However, the incidence, risk factors, and impact on outcome remain controversial.
  
  
  METHODS
  Main clinical, biological, and evolutive features of 82 patients (60 males/22 females; median age: 61 years) diagnosed with MCL (blastoid, 26%) in a single institution were analyzed for risk of CNS involvement and prognosis.
  
  
  RESULTS
  Most patients had advanced stage and intermediate or high-risk International Prognostic Index (IPI). Eleven patients eventually developed CNS involvement with an actuarial 5-year risk of 26% (95% confidence interval 10% to 42%). In one asymptomatic patient, cerebrospinal fluid infiltration was detected at staging maneuvers (1/62; 1.6%). The remaining 10 patients developed neurological symptoms during the course of the disease (median time from diagnosis, 25 months). Initial variables predicting CNS involvement were blastoid histology, high proliferative index measured by Ki-67 staining, high lactate dehydrogenase (LDH) and intermediate- or high-risk IPI. Histological subtype and serum LDH maintained significance in multivariate analysis. Treatment of CNS infiltration consisted of intrathecal chemotherapy (two cases), and intrathecal chemotherapy plus systemic treatment (seven cases). Median survival after CNS involvement was 4.8 months, patients with this complication having shorter survival than those with no CNS disease.
  
  
  CONCLUSIONS
  This study confirms the high incidence of CNS involvement in MCL patients. Treatments aimed at preventing this complication are warranted.
authors.affiliation: Department of Hematology, Hospital Clínic, Postgraduate School of Hematology Farreras Valentí, Institut d'Investigacio Biomedica August Pi i Sunyer, Barcelona, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Ferrer
authors.forename: F
authors.initials: F
authors.lastname: Bosch
authors.forename: N
authors.initials: N
authors.lastname: Villamor
authors.forename: M
authors.initials: M
authors.lastname: Rozman
authors.forename: F
authors.initials: F
authors.lastname: Graus
authors.forename: G
authors.initials: G
authors.lastname: Gutiérrez
authors.forename: S
authors.initials: S
authors.lastname: Mercadal
authors.forename: E
authors.initials: E
authors.lastname: Campo
authors.forename: C
authors.initials: C
authors.lastname: Rozman
authors.forename: A
authors.initials: A
authors.lastname: López-Guillermo
authors.forename: E
authors.initials: E
authors.lastname: Montserrat
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002490:Central Nervous System / Q000473:pathology*; D002555:Cerebrospinal Fluid / Q000166:cytology; D002699:Chlorambucil / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007278:Injections, Spinal; D008218:Lymphocytosis / Q000188:drug therapy / Q000209:etiology; D020522:Lymphoma, Mantle-Cell / Q000134:cerebrospinal fluid / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D011379:Prognosis; D012306:Risk; D012720:Severity of Illness Index; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000970:Antineoplastic Agents; D002699:Chlorambucil; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone; D008727:Methotrexate