Keutgens Aurore, Foguenne Jacques, Gothot André, Tassin Françoise
Laboratoire d'hématologie, CHU Liège, Domaine universitaire du Sart-Tilman B35, Liège, Belgique.
Ann Biol Clin (Paris). 2016 Mar-Apr;74(2):168-75. doi: 10.1684/abc.2016.1127.
Monoclonal B-cell lymphocytosis (MBL) is defined as an asymptomatic condition characterized by the presence of less than 5,000 monoclonal B-cells per microliter and the absence of clinical signs or symptoms of a B-cell lymphoproliferative disorder. Most MBL cases involve B cells presenting an identical phenotype to CLL (CLL-like MBL) with a Catovsky-Matutes score of 3 to 5 and share the same chromosomal abnormalities than CLL. Depending on the absolute B cell count, one may distinguish low-count CLL-like MBL (<500 B cells/μL) which have no evidence of progression, no reduction in overall survival, no increase in infection risk and do not require any specific follow-up. Patients with clinical CLL-like MBL (>500 B cells/μL) have a 1% to 2% per year risk of progression to CLL requiring therapy, a higher risk of infectious complications and mortality implicating an annual follow-up by hematologist. MBL may also express other less common phenotypes and are named atypical MBL in case of CD5 antigen expression (Catovsky-Matutes score: 1-2) and non-CLL-like MBL for CD5 negative cases (Catovsky-Matutes score: 0-2). Their poorer prognosis implicates imaging studies, bone marrow biopsy and cytogenetic analysis in addition to physical examination in order to rule out non-hodgkinien lymphoma, and require a more frequent follow-up. This review focuses on key concepts in the classification, diagnosis, monitoring and biology of MBL in laboratory practice.
单克隆B淋巴细胞增多症(MBL)被定义为一种无症状状态,其特征为每微升存在少于5000个单克隆B细胞,且无B细胞淋巴增殖性疾病的临床体征或症状。大多数MBL病例涉及表现出与慢性淋巴细胞白血病(CLL)相同表型的B细胞(CLL样MBL),其卡托夫斯基-马图茨评分3至5,且与CLL具有相同的染色体异常。根据绝对B细胞计数,可区分低计数CLL样MBL(<500个B细胞/μL),这类患者无进展证据,总生存期无缩短,感染风险无增加,无需任何特殊随访。临床CLL样MBL(>500个B细胞/μL)患者每年有1%至2%的风险进展为需要治疗的CLL,感染并发症和死亡率风险更高,这意味着血液科医生需每年进行随访。MBL也可能表达其他较不常见的表型,CD5抗原表达时(卡托夫斯基-马图茨评分:1 - 2)称为非典型MBL,CD5阴性时(卡托夫斯基-马图茨评分:0 - 2)称为非CLL样MBL。其预后较差意味着除体格检查外,还需进行影像学检查、骨髓活检和细胞遗传学分析以排除非霍奇金淋巴瘤,且需要更频繁的随访。本综述重点关注实验室实践中MBL在分类、诊断、监测和生物学方面的关键概念。