The prognosis of clinical monoclonal B cell lymphocytosis differs from prognosis of Rai 0 chronic lymphocytic leukaemia and is recapitulated by biological risk factors
The prognosis of clinical monoclonal B cell lymphocytosis differs from prognosis of Rai 0 chronic lymphocytic leukaemia and is recapitulated by biological risk factors
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DOI：
10.1111/j.1365-2141.2009.07711.x
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发表时间：
2009-07-01
影响因子：
6.5
通讯作者：
Forconi, Francesco
中科院分区：
文献类型：
--
作者：
Rossi, Davide;Sozzi, Elisa;Forconi, Francesco
单克隆B细胞淋巴细胞增多症（MBL）是一种无症状的单克隆扩增< 5中心点0 × 10（9）/l循环CLL表型B细胞。MBL和Rai 0 CLL之间的关系，以及生物危险因素对MBL预后的影响尚不清楚。在460个具有CLL表型的B细胞扩增中，根据临床和生物学特征和结果，将123个临床MBL（cMBL）与154个Rai 0 CLL进行比较。cMBL具有更好的体液免疫能力和更低的感染风险，del 11 q22-q23/del 17 p13和TP 53突变的发生率更低，淋巴细胞倍增时间更慢，无治疗生存期更长。此外，cMBL诊断是治疗风险的保护因素。尽管有这些有利的特征，所有的cMBL都预计会进展，淋巴细胞< 1个中心点2 x 10（9）/l和> 3个中心点7 x 10（9）/l是预测进展为CLL的最低和最高风险的最佳阈值。尽管IGHV状态、CD 38和CD 49 d表达以及荧光原位杂交（FISH）核型单独预测无治疗生存期，但多变量分析确定+12或del 17 p13的存在是cMBL治疗需求的唯一独立预测因素（风险比：5中心点39，95%置信区间1中心点98-14中心点44，P = 0中心点001）。总体而言，这些数据表明，cMBL具有比Rai 0 CLL更有利的临床病程。鉴于生物学特征可以预测治疗需求，基于生物学指标的分层可能有助于cMBL管理。
P>Monoclonal B-cell lymphocytosis (MBL) is an asymptomatic monoclonal expansion of < 5 center dot 0 x 10(9)/l circulating CLL-phenotype B-cells. The relationship between MBL and Rai 0 CLL, as well as the impact of biological risk factors on MBL prognosis, are unknown. Out of 460 B-cell expansions with CLL-phenotype, 123 clinical MBL (cMBL) were compared to 154 Rai 0 CLL according to clinical and biological profile and outcome. cMBL had better humoral immune capacity and lower infection risk, lower prevalence of del11q22-q23/del17p13 and TP53 mutations, slower lymphocyte doubling time, and longer treatment-free survival. Also, cMBL diagnosis was a protective factor for treatment risk. Despite these favourable features, all cMBL were projected to progress, and lymphocytes < 1 center dot 2 x 10(9)/l and > 3 center dot 7 x 10(9)/l were the best thresholds predicting the lowest and highest risk of progression to CLL. Although IGHV status, CD38 and CD49d expression, and fluorescence in situ hybridization (FISH) karyotype individually predicted treatment-free survival, multivariate analysis identified the presence of +12 or del17p13 as the sole independent predictor of treatment requirement in cMBL (Hazard ratio: 5 center dot 39, 95% confidence interval 1 center dot 98-14 center dot 44, P = 0 center dot 001). Overall, these data showed that cMBL has a more favourable clinical course than Rai 0 CLL. Given that the biological profile can predict treatment requirement, stratification based on biological prognosticators may be helpful for cMBL management.