Malignant hematopoietic cell lines: in vitro models for the study of mantle cell lymphoma

Mantle cell lymphoma (MCL) is a distinct type of B cell malignancy and accounts for approximately 5-10% of non-Hodgkin's lymphomas (NHL). The characteristic cytogenetic aberration in MCL is the translocation (11;14)(q13;q32) present in virtually all cases. This rearrangement at the BCL1 locus at 11q13 dysregulates the gene CCND1 following juxtaposition with immunoglobulin heavy chain (IGH) transcriptional enhancers at 14q32 and leading to overexpression of its protein product, cyclin D1, which plays a key role in the control of the cell cycle. Eight continuous cell lines (plus several sister cell lines) have been hitherto established from lymph nodes or peripheral blood of patients with MCL (n=5) or with a lymphoma which would nowadays be classified as MCL (n=3). Six of these cell lines carry the specific t(11;14) translocation and a seventh cell line while being negative for t(11;14) shows a rearranged BCL1 locus and cyclin D1 overexpression. Each of these MCL cell lines is unique with regard to its immunophenotypical, additional cytogenetic and functional features. In light of the relatively low frequency of this lymphoma and the poor results of current treatment strategies, the availability of various types of MCL-derived cell lines for immunologic, cytogenetic, molecular and functional studies is expected to illuminate the biology of this disease, which in turn will be hopefully translated into new and better therapies.