Matsuda Masayuki, Gono Takahisa, Shimojima Yasuhiro, Hoshii Yoshinobu, Ikeda Shu-ichi
Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan.
Amyloid. 2003 Jun;10(2):110-6. doi: 10.3109/13506120309041732.
AL amyloidosis is an intractable disease resulting from a plasma cell dyscrasia which has a wide clinical spectrum. To investigate the phenotype of plasma cells in the bone marrow, a flow cytometric analysis was carried out in 10 patients with this disease (mean age, 57.8 +/- 7.9 years) and controls with M-protein (positive controls, n = 4) and without it (negative controls, n = 8). All patients were shown to have either A kappa- or A lambda-immunoreactive amyloid deposits on the biopsied tissues. On flow cytometry CD38++CD19+CD56- cells (polyclonal plasma cells) showed no significant difference between patients (0.59 +/- 0.37%) and either negative (2.25 +/- 2.84%) or positive controls (0.38 +/- 0.20%), while CD38++CD19-CD56+ cells (monoclonal plasma cells) showed a significantly higher level in the patients (1.34 +/- 1.54%) than in either negative (0.041 +/- 0.004%, p < 0.005) or positive controls (0.11 +/- 0.09%, p < 0.05). With respect to maturation of plasma cells, five of the patients (50%), three of the positive controls (75%) and all of the negative controls showed a dominant proliferation of mature subtype (CD45+MPC-1+CD49e- or CD45+MPC-1+CD49e+). Immature (CD45+MPC-1- or CD45-MPC-1-) and intermediate (CD45-MPC-1+CD49e-) subtypes were dominantly present in the bone marrow in 2 and 3 patients, respectively. In AL amyloidosis monoclonal plasma cells producing M-protein can be easily and reliably detected in the bone marrow by flow cytometry. This analysis might provide plasma cell phenotypic markers useful for assessing the prognosis and for monitoring the response to treatment.
AL淀粉样变性是一种由浆细胞发育异常导致的难治性疾病,临床谱广泛。为研究骨髓中浆细胞的表型,对10例该疾病患者(平均年龄57.8±7.9岁)以及有M蛋白的对照者(阳性对照,n = 4)和无M蛋白的对照者(阴性对照,n = 8)进行了流式细胞术分析。所有患者活检组织均显示有κ或λ免疫反应性淀粉样沉积物。流式细胞术检测显示,CD38++CD19+CD56-细胞(多克隆浆细胞)在患者(0.59±0.37%)与阴性对照(2.25±2.84%)或阳性对照(0.38±0.20%)之间无显著差异,而CD38++CD19-CD56+细胞(单克隆浆细胞)在患者中的水平(1.34±1.54%)显著高于阴性对照(0.041±0.004%,p < 0.005)和阳性对照(0.11±0.09%,p < 0.05)。关于浆细胞的成熟情况,5例患者(50%)、3例阳性对照者(75%)和所有阴性对照者均显示成熟亚型(CD45+MPC-1+CD49e-或CD45+MPC-1+CD49e+)占优势增殖。不成熟(CD45+MPC-1-或CD45-MPC-1-)和中间(CD45-MPC-1+CD49e-)亚型分别在2例和3例患者的骨髓中占优势。在AL淀粉样变性中,通过流式细胞术可在骨髓中轻松、可靠地检测到产生M蛋白的单克隆浆细胞。该分析可能提供有助于评估预后和监测治疗反应的浆细胞表型标志物。