期刊
LEUKEMIA
卷 22, 期 5, 页码 1044-1052出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/leu.2008.4
关键词
translocation; deletion; p53; cyclin D; RAS; MYC
资金
- NCI NIH HHS [R01 CA083724, P01 CA062242-13, CA21115-25C, P01 CA062242, U10 CA021115, P50 CA100707, P50 CA100707-060008, R01 CA83724-01, P01 CA62242] Funding Source: Medline
- NIA NIH HHS [R01 AG020686-05, R01 AG020686] Funding Source: Medline
Plasma cell leukemia (PCL) is an aggressive and rare hematological malignancy that originates either as primary disease (pPCL) or as a secondary leukemic transformation (sPCL) of multiple myeloma (MM). We report here the genetic aberrations and survival of 80 patients with pPCL or sPCL and make comparisons with 439 cases of MM. pPCL presents a decade earlier than sPCL (54.7 vs 65.3 years) and is associated with longer median overall survival (11.1 vs 1.3 months; P < 0.001). 14q32 (IgH) translocations are highly prevalent in both sPCL and pPCL (82-87%); in pPCL IgH translocations almost exclusively involve 11q13 (CCND1), supporting a central etiological role, while in sPCL multiple partner oncogenes are involved, including 11q13, 4p16 (FGFR3/MMSET) and 16q23 (MAF), recapitulating MM. Both show ubiquitous inactivation of TP53 (pPCL 56%; sPCL 83%) by coding mutation or 17p13 deletion; complemented by p14ARF epigenetic silencing in sPCL (29%). Both show frequent N-RAS or K-RAS mutation. Poor survival in pPCL was predicted by MYC translocation (P = 0.006). Survival in sPCL was consistently short. Overall pPCL and sPCL are different disorders with distinct natural histories, genetics and survival.
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