The clinical outcome of 65 cases of mantle cell lymphoma initially treated with non‐intensive therapy by the British National Lymphoma Investigation Group

Abstract

Mantle cell lymphoma (MCL) was first described as a distinct biological entity on the basis of its association with the t(11;14)(q13;q32) resulting in over‐expression of the cyclin D1 gene. Recognition of the morphological, immunophenotypic and clinical characteristics of MCL has enabled the accurate diagnosis of this entity and appreciation of its poor prognosis. Most published series of patients with MCL have used anthracycline‐containing regimens. In contrast the British National Lymphoma Investigation (BNLI) group have treated 65 patients with MCL with non‐intensive ‘low‐grade lymphoma’ therapy. The median overall survival of 57 months and progression‐free survival of 24 months compares favourably with the more intensively treated series. Although the disease was generally more aggressive than other low‐grade lymphomas, some patients were asymptomatic and had indolent disease. When compared to 1853 patients with non‐MCL low‐grade lymphomas entered on the BNLI database, patients were found on average to be older (P = 0.02), to have more extra‐nodal disease (P < 0.00001), and a higher proportion to have a raised ESR (P = 0.02) and a low serum albumin (P = 0.002). Multivariate analysis of significant prognostic markers in all BNLI low‐grade lymphomas failed to identify MCL as an independent prognostic factor.

Peripheral blood stem cell contamination in mantle cell non-Hodgkin lymphoma: the case for purging?

“…However, the results achieved using this form of treatment, although interesting, have not to date proven superior to those achieved with more conventional treatment. 6,7 Moreover, some discrepancies can be found between reports concerning achievement of long-term disease-free survival (DFS), projected overall survival, varying from 24% 16 at 3 years to 62% at 4 years, 14 based on small numbers of patients. It is worth mentioning that in the latter study, in vitro purging of the graft was used, which raises the question, also alluded to by other authors, 15,18 of whether patients transplanted with a graft free (or PCR-free) of lymphoma cells, will achieve prolonged DFS.…”

“…4,5 It is recognized as an incurable disease by conventional therapy, with a rather poor long-term survival. [6][7][8] High-dose chemo-radiotherapy (HDCT) with autologous stem cell transplantation (ASCT) is a potentially curative modality for aggressive NHL, 9,10 and many investigators have focused on this type of treatment to try to improve the outcome of MCL. The patients are usually given several courses of chemotherapy, sometimes followed by a more intensive regimen, both to further reduce the tumour burden and to mobilize peripheral blood progenitor cells (PBPC) using growth factors.…”

Mantle Cell Lymphoma: Decision Making for Transplant

“…Retrospective analyses of administration of standard chemotherapy have not shown any improvement in overall survival in patients with MCL 6,37 . Most regiments induce around 80% response rates with complete remissions up to 30% of cases 6,8 . While more aggressive regimens were used 38 , reliable cure rates with conventional treatment has not been reported until now 36 .…”

“…At the time of diagnosis, patients tend to have more extranodal disease and low serum albumin 6 . Although MCL has differential diagnosis with Chronic Lymphocytic Leukemia (CLL) and low-grade NHL, mimicking malignancies with indolent behavior, it follows an aggressive course with 10% to 15% long-term survivors 7 despite administration of standard chemotherapy courses commonly used in NHLs 8 , corresponding to a median survival of 3 to 5 years.…”

Stem cell transplantation for mantle cell lymphoma: should it ever be used outside clinical trials?

“…Secondly, the poor prognosis for patients with MCL treated with 'conventional'-dose chemotherapy has been reported in multiple series. [18][19][20][21][22][23][24][25][26][27][28][29][30][31] The reported median overall survival for patients with this disease is only 3 to 4 years, with less than 15% of patients alive at 5 years. As a result, many centers have investigated the use of high-dose therapy in an attempt to overcome the apparent chemo-refractoriness of this disease.…”