Mantle cell lymphoma (MCL)
MCL has features of both indolent and aggressive NHL and accounts for approximately 5% of all lymphoma cases in North America and 8-10% of cases in Europe. It usually grows slowly, but responds poorly to therapy with usually short-lasting remissions. Although formerly classified as an entity belonging to the follicular lymphomas, MCL is now regarded as an entity on its own and has a rather aggressive course. MCL occurs more frequently in men than women (3:1 ratio), usually presenting as generalised lymphadenopathy with, or without, a leukaemic blood profile. Patients with leukaemic MCL appear to have a significantly worse outcome compared to the overall MCL group1. Involvement of other organs is not uncommon, as the disease is often advanced by the time of diagnosis. The most common extranodal sites are the gastrointestinal tract and Waldeyer's ring (20-30% of cases). Involvement of the liver is also common2. With standard treatment regimens, median survival for patients with MCL is approximately 3 years1.
MCL is characterised by the proliferation of atypical small lymphoid cells in the mantle zones around normal germinal centres. The spleen is often enlarged with numerous lymphoid nodules, and microscopically, there is enlargement of the white pulp areas. The tumour cells express B-cell antigens CD19, CD20, CD22 and CD24 and also express CD5 and CD432. Staining for the T-cell antigens CD5 and CD43 allows differentiation of MCL from MZL and FL. The characteristic cytogenetic abnormality in MCL is the t(11;14) (q13;q32) translocation seen in approximately 60% of cases1.
References
- Hiddemann W, et al. In: Non-Hodgkin's lymphomas. Mauch P, Armitage J, Coiffier B, Dalla-Favera R, Harris N, ed. Lippincott Williams & Wilkins 2004:461-76.
- Weisenberger DD, JA. In: Hancock BWSP, McLennan K, Armitage Jo, ed. Malignant Lymphoma.London:Arnold, 2000.
