Lymphoplasmacytic LymphomaとPAX-5

抄録

The t (9; 14) (p13; q32) chromosomal translocation is specifically observed in B-cell malignancies such as approximately an half of lymphoplasmacytic lymphoma (LPL) and a small fraction of diffuse large B-cell lymphoma (DLBCL). It results in the juxtaposition of PAX-5 gene, which encodes for an essential transcription factor (BSAP: B-cell lineage specific activator protein) for B-cell development, to the Ig heavy chain gene (IgH) locus. Deregulated expression of the PAX-5 gene as a consequence of this alteration is assumed to lead to the transformation of the B cells at the lymphoplasmacytic stages, although the mechanisms involved in this process remain unknown. A diagnostic procedure using FISH (fluorescence in situ hybridization), which is capable of detecting 80% of the widely scattering 9p13 breakpoints involved in this translocation, was also developed. Thus, further accumulation of the LPL cases and the understanding of the physiological and the pathological roles of the PAX-5 gene in B-cell development may lead to the establishment of the optimal clinical treatment strategy for LPL and LPL-derived DLBCL cases.