背景:很少报道CD10和BCL-6共表达的套细胞淋巴瘤(MCL),其生物学特征在很大程度上仍然未知。因此,这项研究旨在描述临床和生物学特征,以及CD10和BCL-6共表达的MCL患者的预后。
方法:选取我院2011年1月至2018年10月收治的MCL患者104例。这些患者根据2016年世界卫生组织(WHO)的造血和淋巴组织肿瘤分类系统进行诊断,符合细胞形态学和免疫组织化学分析的结果。通过电话采访对患者进行了随访,医疗记录。年龄差异,性别,白细胞计数,乳酸脱氢酶(LDH)水平,分析β-2微球蛋白(β2-MG)水平及免疫组化结果。然后用Kaplan-Meier法和log-rank检验进行无事件生存率(EFS)和总生存率(OS)。
结果:结果表明,总的来说,5例患者浅表淋巴结肿大,2例患者腹部不适,1例患者的红细胞(RBC)计数在诊断时异常。所有患者均处于IV期,75%有骨髓受累,75%和100%的患者LDH和β2-MG水平升高,分别。这8例患者涉及3个经典基因和5个变异。CD10和BCL-6共表达的MCL患者Ki-67指数较高,白细胞(WBC)计数,LDH水平,和β2-MG水平高于无CD10和BCL-6共表达的水平(P分别为0.025、0.038、0.015和0.021)。此外,CD10和BCL-6共表达的MCL患者的OS和EFS较短(χ2分别为6.401和5.975;P分别为0.011和0.015)。表明患者易患复杂核型和TP53异常。
结论:CD10和BCL-6共表达的MCL患者更可能有骨髓受累,较高的Ki-67指数,白细胞计数增加,诊断时LDH和β2-MG水平升高,然后可能有复杂的细胞遗传学和不良预后。
BACKGROUND: Mantle cell lymphoma (MCL) with co-expression of CD10 and BCL-6 was scarcely reported, and its biological features were largely remained unknown. Thus, this
study aimed to describe the clinical and biological features, as well as outcome of MCL patients with co-expression of CD10 and BCL-6.
METHODS: A total of 104 cases of MCL who were admitted to our hospital between January 2011 and October 2018 were recruited. Those patients were diagnosed according to the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues, in compliance with the results of cytomorphology and immunohistochemical analysis. Patients were followed up through telephone interviews, medical records. Differences in age, gender, leukocyte count, lactate dehydrogenase (LDH) level, beta-2 microglobulin (β2-MG) level and results of immunohistochemistry were analyzed. Then the event-free survival (EFS) rate and overall survival (OS) rate were performed by the Kaplan-Meier method and log-rank test.
RESULTS: The results showed that, in total, 5 patients had superficial lymph node enlargement, 2 patients suffered from abdominal discomfort, and 1 patient\'s red blood cell (RBC) count was abnormal at the time of diagnosis. All patients were in stage IV, 75% had bone marrow involvement, and 75% and 100% of patients had elevated levels of LDH and β2-MG, respectively. Three classic genes and five variants were involved in the 8 patients. MCL patients with the co-expression of CD10 and BCL-6 had higher Ki-67 index, white blood cell (WBC) count, LDH level, and β2-MG level than those of without co-expression of CD10 and BCL-6 (P=0.025, 0.038, 0.015, and 0.021, respectively). Besides, MCL patients with CD10 and BCL-6 co-expression had shorter OS and EFS (χ2=6.401 and 5.975; P=0.011 and 0.015, respectively), indicating patients\' susceptibility to get complex karyotype and TP53 abnormality.
CONCLUSIONS: MCL patients with co-expression of CD10 and BCL-6 were more likely to have bone marrow involvement, higher Ki-67 index, increased WBC count, and elevated levels of LDH and β2-MG at the time of diagnosis, then might has complex cytogenetic and poor prognosis.