Pasqualetti P, Casale R
Department of Internal Medicine and Public Health, School of Medicine and Surgery, University of L'Aquila, Italy.
Biomed Pharmacother. 1997;51(2):74-8. doi: 10.1016/s0753-3322(97)87730-x.
The acturial probability of malignant transformation was analyzed in a series of 263 patients with monoclonal gammopathy of undetermined significance (MGUS) over a 15-year period and followed from 5 to 20 years. At a median follow-up of 11.5 years, 157 patients (59.7%) had died of causes unrelated to MGUS, 47 (17.9%) were still alive and presented no increase in monoclonal component, 11 (4.1%) presented an increase in monoclonal component without evidence of malignant immunoproliferative disease, and 48 (18.3%) had developed a malignant transformation of MGUS. In particular, MGUS evolved into 35 cases of multiple myeloma, two of solitary plasmacytoma of the bone, four of macroglobulinemia, three of malignant lymphoma, two of amyloidosis, one of chronic lymphocytic leukemia, and one of plasma cell leukemia. The cumulative incidence of malignant transformation was 18.3%; and the actuarial risk of malignant transformation was 6.1, 15.4, and 31.3% at 5, 10 and 20 years, respectively. The multivariate regression analysis according to Cox's proportional hazard model selected among 22 different variables established at initial diagnosis of MGUS only age as the factor significantly (P < 0.011) and negatively (b = -1.104) related to the risk of developing a malignant immunoproliferative disease. Therefore, patients with MGUS present an increased risk of developing a malignant lymphoproliferative or plasma cell proliferative disease, and MGUS could be considered a pre-neoplastic condition. Since no clinical or laboratory features are able to identify in advance the patients at high risk of disease progression, each patient must be followed up periodically and over an indefinite period.
对263例意义未明的单克隆丙种球蛋白病（MGUS）患者进行了为期15年的分析，随访时间为5至20年，分析了其恶变的精算概率。中位随访11.5年时，157例患者（59.7%）死于与MGUS无关的原因，47例（17.9%）仍存活且单克隆成分未增加，11例（4.1%）单克隆成分增加但无恶性免疫增殖性疾病证据，48例（18.3%）发生了MGUS恶变。具体而言，MGUS演变为35例多发性骨髓瘤、2例骨孤立性浆细胞瘤、4例巨球蛋白血症、3例恶性淋巴瘤、2例淀粉样变性、1例慢性淋巴细胞白血病和1例浆细胞白血病。恶变的累积发生率为18.3%；恶变的精算风险在5年、10年和20年时分别为6.1%、15.4%和31.3%。根据Cox比例风险模型进行的多变量回归分析，在MGUS初诊时确定的22个不同变量中，仅选择年龄作为与发生恶性免疫增殖性疾病风险显著相关（P<0.011）且呈负相关（b=-1.104）的因素。因此，MGUS患者发生恶性淋巴增殖性或浆细胞增殖性疾病的风险增加，MGUS可被视为一种肿瘤前状态。由于没有临床或实验室特征能够提前识别疾病进展高危患者，因此必须对每位患者进行定期且无期限的随访。
