Division of Pediatrics, Department of Reproductive and Developmental Medicine, University of Miyazaki, Japan.
Am J Hematol. 2011 Jan;86(1):75-8. doi: 10.1002/ajh.21887.
Anaplastic lymphoma kinase (ALK) positive anaplastic large cell lymphoma (ALCL) is usually associated with a favorable prognosis. We describe an 11-year-old girl patient with ALK positive ALCL bearing t(2;5)(p23;q35) and t(8;17)(q24;q25) translocations who had an aggressive clinical course despite various combinations of intensive chemotherapy. Southern blot analysis identified C-MYC rearrangement. Immunohistochemistry and Northern and Western blot analyses revealed cmyc overexpression. A new fusion between ALO17 (ALK lymphoma oligomerization partner on chromosome 17) and C-MYC was identified by the 50-rapid amplification of cDNA ends. This new fusion may have possibly provoked the poor prognosis in this patient with ALK positive ALCL, and C-MYC rearrangement may indicate poor prognosis in ALCL.
间变性淋巴瘤激酶(ALK)阳性间变大细胞淋巴瘤(ALCL)通常与良好的预后相关。我们描述了一例 11 岁 ALK 阳性 ALCL 患者,其携带 t(2;5)(p23;q35) 和 t(8;17)(q24;q25) 易位,尽管接受了各种强化化疗组合治疗,但仍具有侵袭性临床病程。Southern 印迹分析确定了 C-MYC 重排。免疫组织化学和 Northern 和 Western blot 分析显示 cmyc 过表达。通过 50-快速扩增 cDNA 末端鉴定到 ALO17(染色体 17 上的 ALK 淋巴瘤寡聚化伴侣)和 C-MYC 之间的新融合。这种新的融合可能导致该患者 ALK 阳性 ALCL 预后不良,而 C-MYC 重排可能预示着 ALCL 的预后不良。