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.
Local radiotherapy (RT) alone was compared with radiotherapy plus continuous oral chlorambucil (RT+CHL) for the treatment of localised, low grade non-Hodgkins lymphoma (NHL) in a prospective randomised study of 148 patients. After a maximum of 18 years follow up there was no significant difference in overall survival or disease free survival between the two treatment groups. Age greater than 50 years and low serum albumin at diagnosis correlated with a poor prognosis in the series overall. Over one third of patients with localised, low grade NHL may be cured by RT alone and adjuvant chlorambucil as initial therapy confers no survival advantage.
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