作者
RE Tiedemann, N Gonzalez-Paz, RA Kyle, R Santana-Davila, T Price-Troska, SA Van Wier, WJ Chng, RP Ketterling, MA Gertz, K Henderson, PR Greipp, A Dispenzieri, MQ Lacy, SV Rajkumar, PL Bergsagel, AK Stewart, R Fonseca
发表日期
2008/5
期刊
Leukemia
卷号
22
期号
5
页码范围
1044-1052
出版商
Nature Publishing Group
简介
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 …
引用总数
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