Simon Z, Sipka S, Gergely L, Csípö I, Illés A
3rd Department of Internal Medicine, Institute of Internal Medicine, University of Debrecen Medical and Health Science Center, Debrecen, Hungary.
Clin Lab Haematol. 2006 Jun;28(3):164-9. doi: 10.1111/j.1365-2257.2006.00776.x.
Summary The main characteristic of monoclonal gammopathies (MG) is the presence of an increased amount of both electrophoretically and immunologically homogeneous immunoglobulins (M component). According to the WHO classification, monoclonal gammopathies are classified among the non-Hodgkin's lymphomas as 'plasma cell dyscrasias'. The unknown behaviour state, so-called MGUS (monoclonal gammopathy of undetermined significance), is distinguished from the malignant diseases. We investigated the frequency and features of MG and MGUS by reviewing the serum immunochemistry protein analyses between 1998 and 2004. Among 18,642 analyses, MG was found in 1,983 cases (10.39%) derived from one or more samples of 416 patients. Case histories of 340 patients were analysed. A malignant lymphoproliferative disease was proved in 171 cases, while in 169 cases the behaviour of the gammopathy was unknown. In 65 cases, the disease was possibly not related to MG. Mean follow-up time of the 65 patients with MGUS was 42 (9-81) months. During the follow-up period seven patients progressed into a malignant lymphoproliferative disorder-- mean probability of the malignant transformation was 3.07%/year and it occurred more frequently in the presence of immunoglobulin A isotype. There was no correlation between the progression of the disease and other laboratory findings. Besides the analyses of MG-related diseases, we focus on the malignant transformation of MGUS and on the importance of regular clinical and laboratory control examinations.
摘要 单克隆丙种球蛋白病(MG)的主要特征是电泳和免疫方面均一的免疫球蛋白(M成分)含量增加。根据世界卫生组织的分类,单克隆丙种球蛋白病在非霍奇金淋巴瘤中被归类为“浆细胞发育异常”。未知行为状态的所谓意义未明的单克隆丙种球蛋白病(MGUS)与恶性疾病相区分。我们通过回顾1998年至2004年期间的血清免疫化学蛋白分析,研究了MG和MGUS的发生率及特征。在18642次分析中,在416例患者的一个或多个样本中发现1983例(10.39%)MG。分析了340例患者的病历。171例被证实患有恶性淋巴增殖性疾病,而169例丙种球蛋白病的行为状态未知。65例中,疾病可能与MG无关。65例MGUS患者的平均随访时间为42(9 - 81)个月。随访期间,7例进展为恶性淋巴增殖性疾病,恶性转化的平均概率为每年3.07%,且在免疫球蛋白A同种型存在时更频繁发生。疾病进展与其他实验室检查结果之间无相关性。除了分析与MG相关的疾病外,我们重点关注MGUS的恶性转化以及定期临床和实验室对照检查的重要性。