[Long-term remission after CHOP therapy in a case of multifocal extranodal diffuse large B-cell lymphoma with t(1 ; 14) (p22 ; q32) and rearrangement of bcl-10].

The translocation t(1 ; 14)(p22 ; q32) has been reported only in cases of mucosa-associated lymphoid tissue (MALT) lymphomas. Moreover, bcl-10 is a novel apoptotic signaling gene located at 1p22 and t(1 ; 14)(p22 ; q32) may directly expose bcl-10 to Ig somatic hypermutation. A recent report indicates a pathogenic role of bcl-10 mutation in the progression of MALT lymphomas. In this report, we describe the first case of multiple extranodal diffuse large B-cell lymphoma (DLBCL) with t(1 ; 14)(p22 ; q32). A 70-year-old woman was diagnosed as having DLBCL of multiple extranodal sites (lung, duodenum, colon and kidney). Cytogenetic analysis of a renal lesion revealed the chromosome translocations t(1 ; 14)(p22 ; q32) and both IgH and bcl-10 gene rearrangements were confirmed by Southern blot hybridization. The patient received a regimen of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP). She achieved complete remission after six cycles of chemotherapy and has been free of disease for more than five years. This is the first case of bcl-10 gene rearrangement in DLBCL with t(1 ; 14)(p22 ; q32) and this gene may be involved in the pathogenesis of aggressive lymphomas such as MALT lymphomas or in the progression of MALT lymphomas.