Burkitt leukaemia/lymphoma: R-CODOX-M/R-IVAC remains gold standard treatment in BL.

Abstract

Sporadic Burkitt lymphoma (BL), characterised by translocation-associated C-MYC upregulation is a rare, aggressive lymphoma with a cure rate up to 90% using the R-CODOX-M/R-IVAC (RCRI) protocol. RCRI is active in HIV-associated BL in combination with HAART. The WHO classification system defines lymphomas intermediate between DLBCL and BL, in which lymphomas with t(14;18)(q32;q21) and C-MYC-carrying translocation, i.e. 'double-hit' are included (BL-DH), and these patients are conventionally treated with RCRI. We describe the SJH experience of 25 patients with BL, BL+HIV and BL-DH treated with RCRI between 2002 and 2011. Twelve BL patients (8M/4F), median age 49.1years (range 20-73years); of whom 9 had extensive disease, including 8 with marrow and 2 with CNS involvement. Eleven patients remain in remission at 80.5months (range 37-147months) from completion of treatment and one died of progressive BL giving an OS of 91.6% at 1year with no late relapses. Eight patients with BL+HIV were treated (6M/2F) with a median age 40.25years (range 24-64). Five remain in complete remission (CR) at 65months (range 13-109months), three patients died, two of progressive disease and one of treatment-associated hepatotoxicity in CR. Five patients with BL-DH were included; (3M/2F), age 47.8years (range 42-55years); and all patients died of progressive disease, 4 on RCRI therapy and a further patient despite an allogeneic transplantation. These results confirm that RCRI is an effective treatment in adults with BL and BL+HIV and remains the gold standard against which other regimens should be compared. We confirm the poor prognosis found in BL-DH, indicating new treatment approaches are needed for this sub-group which should be identified at diagnosis by FISH analysis.