Greipp P R
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester MN 55905.
Blood Rev. 1989 Dec;3(4):222-36. doi: 10.1016/0268-960x(89)90030-1.
When a patient presents with monoclonal gammopathy, a wide variety of clinical conditions must be considered. The importance of distinguishing accurately between patients with stable monoclonal gammopathies and those with overt multiple myeloma cannot be over-emphasised. The bone marrow examination with plasma cell labeling index, and newer techniques such as magnetic resonance imaging and computed tomography can improve diagnostic discrimination. In difficult cases, the detection of small numbers of circulating myeloma cells, the peripheral blood B-cell labeling index, and light chain isotype suppression may bring better diagnostic resolution. These tests may also be used to help assess disease activity. If the diagnosis is multiple myeloma, prediction of outcome assumes clinical importance. There are widely disparate survivals among patients with different clinical presentations. Standard clinical assays or a combination of these as in clinical staging do not provide sufficient prediction of outcome but are routinely available and therefore widely used. Independent predictive tests such as the plasma cell labeling index and beta 2-microglobulin improve prognostic accuracy. Ploidy analysis and immunophenotyping are additional variables that may assume more importance as the results of ongoing studies appear. Other promising approaches include detection of oncogene and multiple drug resistance gene expression. All such techniques will become more relevant as we apply more intensive treatment earlier in the disease course, particularly for the younger myeloma patients in whom the prognosis is poor.
当患者出现单克隆丙种球蛋白病时，必须考虑多种临床情况。准确区分稳定型单克隆丙种球蛋白病患者和显性多发性骨髓瘤患者的重要性无论如何强调都不为过。骨髓检查结合浆细胞标记指数以及磁共振成像和计算机断层扫描等新技术可以提高诊断的辨别力。在疑难病例中，检测少量循环骨髓瘤细胞、外周血B细胞标记指数和轻链同型抑制可能会带来更好的诊断分辨率。这些检查也可用于帮助评估疾病活动度。如果诊断为多发性骨髓瘤，对预后的预测就具有临床重要性。不同临床表现的患者生存情况差异很大。标准临床检测或如临床分期中这些检测的组合并不能充分预测预后，但因常规可用所以被广泛使用。诸如浆细胞标记指数和β2-微球蛋白等独立预测检测可提高预后准确性。倍体分析和免疫表型分析是另外的变量，随着正在进行的研究结果出现可能会变得更加重要。其他有前景的方法包括检测癌基因和多药耐药基因表达。随着我们在疾病进程中更早地应用更强化的治疗，尤其是对于预后较差的年轻骨髓瘤患者，所有这些技术将变得更具相关性。
