在慢性淋巴细胞白血病中，SLAMF1 失调与基因组复杂性相关，并独立预测预后不良。

In chronic lymphocytic leukaemia, SLAMF1 deregulation is associated with genomic complexity and independently predicts a worse outcome.

机构信息

Hematology Section, Department of Medical Sciences, Azienda Ospedaliero-Universitaria, Arcispedale S. Anna, University of Ferrara, Ferrara, Italy.

Department of Morphology, Surgery and Experimental Medicine, and "Laboratorio per le Tecnologie delle Terapie Avanzate" (LTTA), University of Ferrara, Ferrara, Italy.

出版信息

Br J Haematol. 2021 Mar;192(6):1068-1072. doi: 10.1111/bjh.16865. Epub 2020 Jun 24.

DOI:10.1111/bjh.16865

PMID:32578873

Abstract

In a series of 349 patients with chronic lymphocytic leukaemia (CLL), we found lower levels of signalling lymphocytic activation molecule family member 1 (SLAMF1) expression in cases with highly complex karyotypes, as defined by the presence of five or more chromosomal abnormalities (CK5; P < 0·001) and with major chromosomal structural abnormalities (P < 0·001). SLAMF1 downregulation was significantly associated with advanced Binet Stage (P = 0·001), CD38 positivity (P < 0·001), high β -microglobulin levels (P < 0·001), immunoglobulin heavy chain variable region gene (IGHV) unmutated status (P < 0·001), 11q deletion (P < 0·001), tumour protein p53 (TP53) disruption (P = 0·011) and higher risk CLL International Prognostic Index categories (P < 0·001). Multivariate analysis showed that downregulated SLAMF1 levels had independent negative prognostic impact on time-to-first treatment (P < 0·001) and overall survival (P < 0·001).

摘要

在 349 例慢性淋巴细胞白血病（CLL）患者中，我们发现具有高度复杂核型的病例中信号淋巴细胞激活分子家族成员 1（SLAMF1）的表达水平较低，定义为存在五种或更多染色体异常（CK5；P<0·001）和主要染色体结构异常（P<0·001）。SLAMF1 的下调与晚期 Binet 分期（P=0·001）、CD38 阳性（P<0·001）、β-微球蛋白水平升高（P<0·001）、免疫球蛋白重链可变区基因（IGHV）未突变状态（P<0·001）、11q 缺失（P<0·001）、肿瘤蛋白 p53（TP53）破坏（P=0·011）和更高风险的 CLL 国际预后指数类别（P<0·001）显著相关。多变量分析显示，下调的 SLAMF1 水平对首次治疗时间（P<0·001）和总生存时间（P<0·001）具有独立的负预后影响。