Strategic Research Program on CLL, Università Vita-Salute San Raffaele and IRCCS Ospedale San Raffaele, Milano, Italy.
Strategic Research Program on CLL, Università Vita-Salute San Raffaele and IRCCS Ospedale San Raffaele, Milano, Italy.
Eur J Intern Med. 2018 Dec;58:2-6. doi: 10.1016/j.ejim.2018.09.006. Epub 2018 Sep 26.
Monoclonal B-cell lymphocytosis (MBL) is defined by the presence of a monoclonal B-cell population in the peripheral blood (PB) at a concentration of <5 × 10/l and no signs or symptoms of a lymphoproliferative disorder. In around 75% of cases, the immunophenotype of the clonal B-cell expansions is superimposable to that of chronic lymphocytic leukemia (CLL), thus defined "CLL-like". Other cases may coexpress CD19, CD5, bright CD20, and lack CD23 ("atypical CLL"), while others are CD5-negative ("non-CLL"). Beside the immunophenotypic profile, a key distinction is based on the B-cell count, stratifying the MBL category in low (<0.5 × 10/l) or high-count (≥0.5 × 10/l). Low-count (LC) MBL is recognized in general population studies and it is not associated with lymphocytosis. High-count (HC) MBL is identified during the clinical work-up for lymphocytosis and carries a risk of progression to CLL requiring therapy of 1-2% per year in most series, warranting clinical monitoring over time. At the time of MBL diagnosis, the key point is the careful evaluation and exclusion of differential diagnoses. After the initial workup, the follow-up at a referral center by a hematologist would be desirable as, in addition to the obvious risk of progression to clinically relevant CLL, the appropriate management of MBL individuals should take into account the risk of developing infections, other cancers and autoimmune disorders. For those cases who indeed bear a risk, though limited, of clinical consequences, such as the majority of HC-MBL cases, current evidences suggest that they may benefit from a tailored and specialized approach.
单克隆 B 细胞淋巴增生症(MBL)是指外周血(PB)中存在单克隆 B 细胞群体,浓度<5×10/l,且无淋巴增生性疾病的体征或症状。在大约 75%的病例中,克隆性 B 细胞扩增的免疫表型与慢性淋巴细胞白血病(CLL)重叠,因此被定义为“CLL 样”。其他病例可能同时表达 CD19、CD5、明亮的 CD20,并且缺乏 CD23(“非典型 CLL”),而其他病例则为 CD5 阴性(“非 CLL”)。除了免疫表型特征外,一个关键区别是基于 B 细胞计数,将 MBL 分类为低计数(<0.5×10/l)或高计数(≥0.5×10/l)。低计数(LC)MBL 在一般人群研究中被识别,与淋巴细胞增多无关。高计数(HC)MBL 在淋巴细胞增多的临床评估中被识别,并且在大多数系列中每年有 1-2%的进展为 CLL 的风险,需要治疗,因此需要随着时间的推移进行临床监测。在 MBL 诊断时,关键点是仔细评估和排除鉴别诊断。在初始检查后,如果可能,应由血液科医生在转诊中心进行随访,因为除了明显进展为具有临床意义的 CLL 的风险外,MBL 患者的适当管理还应考虑到发生感染、其他癌症和自身免疫性疾病的风险。对于那些确实存在有限临床后果风险的病例,如大多数 HC-MBL 病例,目前的证据表明,他们可能受益于量身定制的专业方法。