A novel recurrent translocation t(11;14)(p11;q32) in splenic marginal zoneB cell lymphoma

A novel recurrent translocation t(11;14)(p11;q32) was found in three patien ts with splenic marginal zone B cell lymphoma (MZBCL). Fluorescence in situ hybridization (FISH) studies with IgH probes revealed in all cases involve ment of the IgH locus, with breakpoint downstream of the IGV(H) sequences. Partner genes at 11p11 were not identified. The translocation defined the s tem line in two patients, who carried additional cytogenetic aberrations, i ncluding a 17p deletion, present in both cases. In one patient a 7q- chromo some was the primary cytogenetic defect, the t(11;14) having been found in four out of 11 abnormal metaphase cells at the time of transformation Into high-grade MZBCL. Hematological features in all cases included splenomegaly with peripheral blood (PB) involvement by a monoclonal B cell population c onsisting of lymphocytes with villous projections and several blast-like ce lls. The immunophenotype was CD19(+); CD22(bright+); CD23(-), CD10(-), CD5( -), surface Ig(bright+). A bone biopsy in one patient revealed an interstit ial infiltration with an intrasinusoidal pattern of growth. Histological st udies on spleen specimens in two patients showed an expanded marginal zone, with small lymphocytes and several blast-like cells. One patient had a the rapy-demanding disease, with partial, short-term responses to cytotoxic tre atment; one patient transformed into a high-grade MZBCL involving the gut, the PB and the bone marrow 2 years after diagnosis; one patient was unrespo nsive to cytotoxic treatment and underwent splenectomy. The t(11;14)(p11;q3 2) may define a subset of splenic MZBCL with a high-grade component and a r elatively aggressive clinical behavior.