Efficacy of four different regimens in 64 mantle-cell lymphoma cases: clinicopathologic comparison with 498 other non-Hodgkin's lymphoma subtypes. European Organization for the Research and Treatment of Cancer Lymphoma Cooperative Group.

PURPOSE: Before recognizing mantle-cell lymphoma (MCL) as a distinct entity, these patients were grouped into low-grade (LG) or intermediate-/high-grade categories (IGHG) according to the Working Formulation and received various therapies. This was a unique opportunity to evaluate characteristics, behavior, response to treatment, and outcome of patients with MCL from two phase III trials conducted by the European Organization for the Research and Treatment of Cancer (EORTC): EORTC 20855 IGHG and EORTC 20856 LG. PATIENTS AND METHODS: After histologic review, 64 diagnosed MCL patients (29 IGHG and 35 LG) were compared with other patients in their respective trials. In the IGHG group, patients received cyclophosphamide, doxorubicin, teniposide (VM26), prednisone, vincristine, and bleomycin (CHVmP-VB) or modified doxorubicin, cyclophosphamide, etoposide (VP 16), mechlorethamine, vincristine, procarbazine, and prednisone (ProMACE-MOPP). In the LG group, after receiving cyclophosphamide, vincristine, and prednisone (CVP) induction, patients were randomized between maintenance treatment with interferon alfa-2a (IFN) or no further treatment. RESULTS: MCL patients compared with IGHG subtypes showed a similar overall survival and response rate, but shorter duration of response and progression-free survival. Comparing with LG patients, their response rate, duration of response, and progression-free survival showed no difference, while their overall survival was nearly twice shorter. MCL patients treated with CHVmP-VB had the longest survival. No treatment showed any significant improvement in terms of progression-free survival. CONCLUSION: These data confirm that MCL represents a clinicopathologic entity. In terms of survival, it behaves like IGHG subtypes, while in terms of progression-free survival, it behaves like LG lymphoma. It is still not clear which first-line treatment offers patients with MCL the best chance to obtain both a complete response (CR) and a long-term survival.

Two Translocations: a Follicular Variant 2; 18 and a Burkitt 8;14 in a Small Non Cleaved Non Hodgkin's Lymphoma.

The t(2;18)(p11;q21) has recently been described in two lymphoma cases as a variant of the t(14;18)(q32;q21) typical chromosome translocation in follicular lymphomas. Molecular investigations of t(2;18) confirmed juxtaposition of the bcl-2 gene to the immunoglobulin kappa (Igk) locus and described a new break point region on 18q21 found also in the recently reported, second follicular variant translocation (18;22)(q21;q11). Thus, cytogenetic and molecular studies established the same mechanism of (onco)gene activation by the heavy or light Ig gene in follicular lymphomas and Burkitt lymphomas. We describe a case of small non cleaved non Hodgkin's lymphoma in which translocation (2;18) coexisted with a typical (8;14) Burkitt translocation. Absent HLA-DR expression by the tumour cells was noted in this case. The possible implications of the cytogenetic and immunologic findings are discussed.