Schaar C G, Ong F, Snijder S, Wijermans P W, Franck P F, Kluin-Nelemans J C
Leids Universitair Medisch Centrum, RC, Leiden.
Ned Tijdschr Geneeskd. 1998 Jul 11;142(28):1591-5.
Diagnoses in patients with paraproteinaemia are diverse; few (mostly single centre based) studies are known that describe incidence, diagnoses and follow-up in patients with paraproteinaemia. In the region of the Comprehensive Cancer Centre West in the Netherlands (population 1.6 million, 1992) a population-based registry was set up in the period 1991-1993. Patients (n = 1464; median age: 72 years; range: 16-102) were entered by clinical chemists, internists, haematologists, and pathologists. Multiple myeloma and plasmacytoma were diagnosed in 261 patients (18%), paraprotein-related haematological diseases in 159 patients (11%) and paraprotein-related internal diseases in 210 patients (14%). After bone marrow examination monoclonal gammopathy of unknown significance (MGUS) was diagnosed in 207 (14%) patients. No further diagnosis could be made in 627 (43%) patients mostly for lack of supplementary bone marrow and (or) X-ray examinations. Consequently, more than two-thirds of all patients with a newly found paraprotein did not show any sign of a haematological malignancy. Using these data a 'myeloma risk score' was developed to predict the presence of a multiple myeloma based on paraprotein type and concentration, aiding the physician in determining which patients should undergo further bone marrow and skeletal examinations.
副蛋白血症患者的诊断多种多样;已知很少有研究(大多基于单中心)描述副蛋白血症患者的发病率、诊断及随访情况。在荷兰西部综合癌症中心所在地区(1992年人口为160万),于1991 - 1993年期间建立了一个基于人群的登记处。患者(n = 1464;中位年龄:72岁;范围:16 - 102岁)由临床化学家、内科医生、血液学家和病理学家录入。261例患者(18%)被诊断为多发性骨髓瘤和浆细胞瘤,159例患者(11%)被诊断为副蛋白相关血液疾病,210例患者(14%)被诊断为副蛋白相关内科疾病。经骨髓检查,207例(14%)患者被诊断为意义未明的单克隆丙种球蛋白病(MGUS)。627例(43%)患者大多因缺乏补充骨髓检查和(或)X线检查而无法做出进一步诊断。因此,新发现副蛋白的所有患者中,超过三分之二未表现出血液系统恶性肿瘤的任何迹象。利用这些数据开发了一个“骨髓瘤风险评分”,以根据副蛋白类型和浓度预测多发性骨髓瘤的存在,帮助医生确定哪些患者应接受进一步的骨髓和骨骼检查。