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ｃ−ＭＹＣ/免疫グロブリン重鎖融合遺伝子を有する非バーキット大細胞型リンパ腫の遺伝子解析と臨床病態：赤坂尚司¹、赤坂浩司¹、大野仁嗣¹、内山卓¹（¹京大・医・血液病態学）

Clinical and molecular features of non-Burkitt, diffuse large cell lymphoma associated with the c-MYC/IGH fusion gene : Takashi AKASAKA¹, Hiroshi AKASAKA¹, Hitoshi OHNO¹, Takashi UCHIYAMA¹ (¹Dept. of Hematology/Oncology, Kyoto Univ.)

t(8;14)(q24;q32) and/or rearrangement of the c-MYC gene have been observed not only in Burkitt lymphoma (BL) but also in a substantial number of cases with non-BL, diffuse large cell lymphoma of B-cell type (DLCL). We have developed the long-distance (LD-) PCR technique to detect the c-MYC/immunoglobulin heavy chain (IGH) fusion gene which is the molecular equivalent of t(8;14) of the sporadic type BL (sBL). Genomic DNA extracted from a total of 204 cases with DLCL were subjected to the LD-PCR analysis and 12 cases (5.9%) showed positive amplification; 1 had a c-MYC/Cμ, 9 had a c-MYC/Cγ and 2 had a c-MYC/Cα fusion sequences. The LD-PCR products, ranging from 2.3 to 12.0 kb in size, were cloned into plasmids and restriction analysis were performed. The results showed that breakage occured upstream of c-MYC or within the exon 1, indicating that the c-MYC/IGH fusion of DLCL is identical to that of sBL at the molecular level. The age range of the 12 patients was 44 to 86 years with a median of 65. Four had a stage I/II disease and 8 had a stage III/IV disease. LDH was elevated in 8 of 10 examined. Seven showed involvement of the gastrointestinal tract. All but one patients were treated with combination chemotherapy; the survival was 64 to 2338+ days and 4 have enjoyed long disease-free survival. These results indicate that DLCL with the c-MYC/IGH fusion gene shows rapidly progressive course, however, the disease is potentially curable with appropriate chemotherapy.