Kanoh T, Hirashimizu K
Department of Internal Medicine, Faculty of Medicine, Kyoto University.
Rinsho Ketsueki. 1993 Apr;34(4):439-43.
The clinical course of multiple myeloma (MM), ranging from relatively asymptomatic form to frankly aggressive neoplasia, is more variable than that of other hematologic malignancies. The nature of tumor cells and/or the secondary effects of malignancy as anemia, hypercalcemia, and renal failure have shown to correlate with clinical behavior of MM. Prognostic variables include age, degree of anemia, morphologic subtypes, serum creatinine and calcium levels, Bence Jones proteinuria, plasma cell LI%, beta 2MG level, nucleolus-associated J chains and other laboratory prognostic factors. The plasma cell LI% is the most reliable predictor of survival. Analysis of the presenting features and the clinical characteristics indicates that there are several variants of MM with a poor prognosis, including juvenile myeloma, plasma cell leukemia, aggressive myeloma, high LDH myeloma, J chain myeloma, and amylase-producing myeloma. Four relapsing patterns have been pointed out. The appearance of an additional M-component (mutation escape) suggests the terminal or advanced stage of illness. A new lambda-type M-component can be found in patients with kappa-type myeloma. The prognostic significance of Bence Jones escape varies for different stage of illness. Bence Jones escape is an important predictor of the development of overt MM in patients with smoldering MM. The need for clearly established prognostic criteria is imperative for the choice of correct therapeutic strategies.
多发性骨髓瘤（MM）的临床病程差异较大，从相对无症状到明显侵袭性肿瘤，比其他血液系统恶性肿瘤的病程变化更大。肿瘤细胞的性质和/或恶性肿瘤的继发效应，如贫血、高钙血症和肾衰竭，已显示与MM的临床行为相关。预后变量包括年龄、贫血程度、形态学亚型、血清肌酐和钙水平、本周氏蛋白尿、浆细胞LI%、β2微球蛋白水平、核仁相关J链及其他实验室预后因素。浆细胞LI%是生存的最可靠预测指标。对首发特征和临床特点的分析表明，MM有几种预后不良的变异型，包括青少年骨髓瘤、浆细胞白血病、侵袭性骨髓瘤、高乳酸脱氢酶骨髓瘤、J链骨髓瘤和产生淀粉酶的骨髓瘤。已指出四种复发模式。额外M成分的出现（突变逃逸）提示疾病的终末期或晚期。κ型骨髓瘤患者可发现新的λ型M成分。本周氏蛋白逃逸的预后意义在疾病的不同阶段有所不同。本周氏蛋白逃逸是冒烟型MM患者发生明显MM的重要预测指标。对于选择正确的治疗策略而言，明确确立预后标准至关重要。
