Central Nervous System (CNS) Relapse In a Population of 143 Patients (Pts) with Mantle Cell Lymphoma (MCL) From Two Centers

Abstract 2660

Inconsistent information concerning the pattern of CNS relapse have been reported in MCL pts. We retrospectively analyzed the clinical variables at diagnosis and outcome, with special reference to CNS relapse, in a population of consecutive pts with confirmed diagnosis of MCL from two hematology centers.

Among 2426 non Hodgkin's lymphoma pts treated from 1979 to 2011, 142 cases (44 female, 98 male) of MCL were selected. Median age at diagnosis was 68 years (17–94 years); 116 pts (82%) had stage III-IV, 89 (67% of the 132 cases in whom the data was available) had intermediate-high/high International Prognostic Index (IPI) risk. Extranodal disease was reported in 127 pts (89%), serum LDH was elevated in 45 pts (40% of 113 tested pts). Information concerning first line treatment was available in 139 pts. Fourteen pts (10%) did not receive active treatment at diagnosis, in 7 (5%) of these, systemic treatment followed the initial expectant strategy. Four pts (3%) only received radiotherapy and/or surgery. One hundred twenty two pts (88%) were treated with chemotherapy, 46 pts (33%) had rituximab, alone (6 pts, 4%) or in combination with chemotherapy. Eighteen pts (13%) received chemotherapy regimens including drugs crossing the blood-brain barrier or prophylactic intrathecal chemotherapy, 10 pts (7%) had autologous stem cell transplantation.

After median follow-up of 8 years, CNS relapse was observed in 11 cases (7.7%; 95%CI:4–13%). CNS disease occurred at a median of 13.8 months (range:3.7–95 months) from diagnosis. Cumulative risk of CNS relapse raises until 10 years, being 7.2% (95%CI:4–14%) at 3 years, 10.6% (95%CI:6–19%) at 5 years and 13.6% (95%CI:7–25%) at 10 years. Elevated serum LDH at diagnosis was significantly associated with higher risk of CNS relapse at univariate analysis (P=0.006). Actuarial risk of CNS relapse was significantly shorter in pts with higher risk according to IPI (P=0.018). Median survival after CNS relapse was 6.3 months (range: 1.5–77 months). CNS relapse had a dismal impact on survival (P=0.04). No specific treatment approach at diagnosis, including autologous stem cell transplantation, intrathecal chemotherapy, high dose cytarabine or rituximab, alone or in combination with chemotherapy, significantly reduced the risk of CNS relapse.

CNS relapse is one of the most challenging events in the management of MCL. Our data confirmed the adverse clinical outcome of MCL after CNS relapse. Better definition of clinico-pathological profile at diagnosis suggesting higher risk of CNS relapse could select pts candidate to prophylactic approach addressed to prevent CNS disease.