Roberts-Thomson Peter J, Nikoloutsopoulos Tony, Smith Anthony J F
Department of Immunology, Allergy and Arthritis, Flinders Medical Centre, Bedford Park, Adelaide, South Australia.
Pathology. 2002 Aug;34(4):356-61. doi: 10.1080/003130202760120535.
To review the disease associations and laboratory features occurring in IgM paraproteinaemia.
Systematic review of all new serum IgM paraproteins detected over a 6-year period in an immunodiagnostic laboratory serving a population of 400,000 people. Clinical diagnoses were ascertained from a computerised laboratory database or clinical notes, whilst associated laboratory features were obtained from the same sources.
The 125 IgM paraproteins detected constitute 19.7% of all new paraproteins observed over the period of study. IgM paraproteinaemia occurred more commonly in males and its frequency increased with age. Approximately 30% were associated with B cell lymphoproliferative disorders (Waldenstrom's macroglobulinaemia, non-Hodgkin's lymphoma, chronic lymphocytic leukaemia, amyloid, etc.) with the remainder being labelled as monoclonal IgM gammopathies of uncertain significance (four having a peripheral neuropathy). At clinical presentation, patients with lymphoproliferative disorders tended to have higher levels of IgM, beta2-microglobulin, the presence of free urinary light chains and demonstrated molecular size heterogenicity of the paraprotein (presence of decamers, oligomers and monomers in addition to the pentamer) but there was considerable overlap. A good correlation was noted between paraprotein concentration and viscosity in most patients.
IgM paraproteinaemia was most frequently encountered in the context of a gammopathy of uncertain significance. Features which suggested lymphoproliferative disorders included higher levels of paraprotein (>15 g/l) elevated levels of beta2-microglobulin and the presence of urinary free high chain. However, as much overlap was seen, regular monitoring of paraprotein levels is considered mandatory in the management of these patients.
回顾IgM副蛋白血症的疾病关联及实验室特征。
对一家为40万人口服务的免疫诊断实验室在6年期间检测到的所有新发血清IgM副蛋白进行系统回顾。临床诊断通过计算机化实验室数据库或临床记录确定,而相关实验室特征则从相同来源获取。
检测到的125种IgM副蛋白占研究期间观察到的所有新发副蛋白的19.7%。IgM副蛋白血症在男性中更常见,其发生率随年龄增加而升高。约30%与B细胞淋巴增殖性疾病(华氏巨球蛋白血症、非霍奇金淋巴瘤、慢性淋巴细胞白血病、淀粉样变等)相关,其余被标记为意义未明的单克隆IgM丙种球蛋白病(4例伴有周围神经病变)。在临床表现上,淋巴增殖性疾病患者的IgM、β2-微球蛋白水平往往较高,存在游离尿轻链,且副蛋白表现出分子大小异质性(除五聚体外还存在十聚体、寡聚体和单体),但有相当大的重叠。大多数患者的副蛋白浓度与黏度之间存在良好相关性。
IgM副蛋白血症最常出现在意义未明的丙种球蛋白病背景下。提示淋巴增殖性疾病的特征包括副蛋白水平较高(>15 g/l)、β2-微球蛋白水平升高以及存在尿游离重链。然而,由于存在大量重叠,在这些患者的管理中定期监测副蛋白水平被认为是必要的。