Ocqueteau M, Orfao A, Almeida J, Bladé J, González M, García-Sanz R, López-Berges C, Moro M J, Hernández J, Escribano L, Caballero D, Rozman M, San Miguel J F
Servicio de Hematología, Hospital Universitario de Salamanca, Spain.
Am J Pathol. 1998 Jun;152(6):1655-65.
Although the immunophenotype of plasma cells (PCs) from multiple myeloma (MM) patients has been extensively explored, information on the phenotypic characteristics of PCs in monoclonal gammopathy of undetermined significance (MGUS) patients is scanty and frequently controversial. Thus, the question of whether or not PCs are phenotypically different in the two disorders and whether this criteria could be useful for the differential diagnosis between MGUS and MM remains to be explored. In the present study, the immunophenotypic profile of bone marrow PCs (BMPCs) from a group of 76 MGUS patients has been analyzed by flow cytometry and compared with that of BMPCs present in both MM patients (n = 65) and control subjects (n = 10). For that purpose, a large panel of monoclonal antibodies against PC-related antigens was used together with a sensitive methodology in which a minimum of 10(3) PCs were studied. In all MGUS cases studied, two clearly defined and distinct PC subpopulations could be identified. One PC subpopulation, population A (33 +/- 31% of total PCs), constantly displayed a high CD38 expression with low forward light scatter (FSC)/side light scatter (SSC) and was positive for CD19 and negative for CD56 (only a small proportion of these PCs were weakly positive for CD56). The other PC subpopulation, population B (67 +/- 31% of total PCs), showed the opposite pattern; the antigen CD56 was strongly positive and CD19 was constantly negative, and it showed a lower CD38 expression and higher FSC/SSC values than population A. Clonality studies (cytoplasmic light chain restriction, DNA content studies, and polymerase chain reaction assessment) confirmed the clonal nature of PCs from population B and the polyclonal origin of PCs from population A. Moreover, the polyclonal PCs from MGUS displayed a phenotypic profile identical to that found in PCs from healthy individuals. By contrast, clonal PCs from all MGUS patients displayed a similar antigenic profile to myelomatous PCs, with clear phenotypic differences with respect to normal PCs: lower intensity of CD38 expression and a variable reactivity for markers that were not expressed in normal PCs, such as CD28, CD117, and sIg. Although the presence of residual polyclonal PCs was a constant finding in MGUS patients, it was a rare event in MM and, when present (only 22% of MM cases), its frequency was significantly lower than that observed in MGUS (0.25% versus 32.9%, respectively; P < 0.0001). Only 1.5% of patients with MM had more than 3% of normal PCs, whereas 98% of patients with MGUS had more than 3%. Moreover, as shown by multivariate analysis, the number of residual polyclonal PCs was the most powerful single parameter for the discrimination between MGUS and MM patients at diagnosis, even when only stage I MM cases were considered.
尽管对多发性骨髓瘤(MM)患者浆细胞(PC)的免疫表型进行了广泛研究,但关于意义未明的单克隆丙种球蛋白病(MGUS)患者PC的表型特征信息却很少,且常常存在争议。因此,这两种疾病中的PC在表型上是否存在差异以及该标准是否有助于MGUS和MM的鉴别诊断仍有待探索。在本研究中,通过流式细胞术分析了一组76例MGUS患者骨髓PC(BMPC)的免疫表型,并与MM患者(n = 65)和对照受试者(n = 10)的BMPC进行了比较。为此,使用了一大组针对PC相关抗原的单克隆抗体以及一种敏感的方法,其中至少研究了10³个PC。在所有研究的MGUS病例中,可以识别出两个明确界定且不同的PC亚群。一个PC亚群,即A群(占总PC的33±31%),始终表现出高CD38表达,前向光散射(FSC)/侧向光散射(SSC)低,CD19阳性,CD56阴性(这些PC中只有一小部分对CD56弱阳性)。另一个PC亚群,即B群(占总PC的67±31%),表现出相反的模式;抗原CD56强阳性,CD19始终阴性,并且其CD38表达低于A群,FSC/SSC值高于A群。克隆性研究(细胞质轻链限制、DNA含量研究和聚合酶链反应评估)证实了B群PC的克隆性质以及A群PC的多克隆起源。此外,MGUS的多克隆PC表现出与健康个体PC相同的表型特征。相比之下,所有MGUS患者的克隆PC表现出与骨髓瘤PC相似的抗原谱,与正常PC存在明显的表型差异:CD38表达强度较低,对正常PC中不表达的标志物(如CD28、CD117和sIg)具有可变反应性。尽管MGUS患者中始终存在残留的多克隆PC,但在MM中这是罕见事件,当存在时(仅占MM病例的22%),其频率明显低于MGUS(分别为0.25%对32.9%;P < 0.0001)。只有1.5%的MM患者正常PC超过3%,而98%的MGUS患者正常PC超过3%。此外,多变量分析表明,残留多克隆PC的数量是诊断时区分MGUS和MM患者的最有力单一参数,即使仅考虑I期MM病例也是如此。