Avet-Loiseau Hervé, Facon Thierry, Grosbois Bernard, Magrangeas Florence, Rapp Marie-José, Harousseau Jean-Luc, Minvielle Stéphane, Bataille Régis
Laboratory and Clinical Department of Hematology, University Hospital, 9 quai Moncousu, 44093 Nantes, France.
Blood. 2002 Mar 15;99(6):2185-91. doi: 10.1182/blood.v99.6.2185.
Multiple myeloma (MM) is a plasma-cell malignancy characterized by marked epidemiological, biological, and clinical heterogeneity. The goal of this study was to find a genetic basis for this heterogeneity. Using fluorescence in situ hybridization, we analyzed a prospective cohort of 901 patients with various plasma-cell disorders--monoclonal gammopathies of undetermined significance, smoldering MM, MM, and primary plasma-cell leukemia--for genetic abnormalities involving the 13q14 and 14q32 chromosomal regions; the patients were consecutively enrolled in the Intergroupe Francophone du Myélome clinical trials, We performed statistical analyses comparing these chromosomal abnormalities in terms of immunological (ie, immunoglobulin types and light-chain subtypes) and clinical status and, to some extent, prognostic features. It was found that 14q32 translocations and del(13) are the most frequent chromosomal abnormalities, observed in 75% and 45% of the patients, respectively, and are not randomly distributed, but interconnected. Second, correlations between them allowed us to define 4 major genetic categories of patients: (1) patients lacking any 14q32 abnormality (25%) and generally also lacking del(13); (2) patients presenting either t(4;14) or t(14;16), almost always associated with a del(13) (15% of patients); (3) patients with other 14q32 abnormalities and presenting del(13) (25%); and (4) patients with other 14q32 abnormalities but not presenting del(13) (35%). Third, we show that this genetic stratification is highly correlated with immunological status and clinical presentation and with some major prognostic factors. For the first time, this study gives genetic support to the heterogeneity observed in patients with MM and demonstrates that the 14q32 and 13q chromosomal abnormalities are not randomly distributed. The strong correlations we found might be the basis for a novel genetic classification of MM, as has been previously demonstrated for leukemias and lymphomas. Furthermore, our study supports different models for MM oncogenesis.
多发性骨髓瘤(MM)是一种浆细胞恶性肿瘤,具有显著的流行病学、生物学和临床异质性。本研究的目的是寻找这种异质性的遗传基础。我们使用荧光原位杂交技术,对901例患有各种浆细胞疾病的患者进行了前瞻性队列分析,这些疾病包括意义未明的单克隆丙种球蛋白病、冒烟型MM、MM和原发性浆细胞白血病,分析涉及13q14和14q32染色体区域的基因异常情况;这些患者连续纳入了法语国家骨髓瘤研究组的临床试验。我们进行了统计分析,比较这些染色体异常在免疫(即免疫球蛋白类型和轻链亚型)和临床状态方面的情况,并在一定程度上比较了预后特征。结果发现,14q32易位和del(13)是最常见的染色体异常,分别在75%和45%的患者中观察到,它们并非随机分布,而是相互关联的。其次,它们之间的相关性使我们能够定义4种主要的患者基因类别:(1)缺乏任何14q32异常的患者(25%),通常也缺乏del(13);(2)出现t(4;14)或t(14;16)的患者,几乎总是伴有del(13)(占患者的15%);(3)有其他14q32异常且出现del(13)的患者(25%);(4)有其他14q32异常但未出现del(13)的患者(35%)。第三,我们表明这种基因分层与免疫状态、临床表现以及一些主要预后因素高度相关。本研究首次为MM患者中观察到的异质性提供了遗传学支持,并证明14q32和13q染色体异常并非随机分布。我们发现的强相关性可能是MM新的基因分类的基础,正如先前在白血病和淋巴瘤中所证明的那样。此外,我们的研究支持MM肿瘤发生的不同模型。