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良性单克隆丙种球蛋白病的生理病理学意义:64例研究

The physiopathological significance of benign monoclonal gammopathy: a study of 64 cases.

作者信息

Carter A, Tatarsky I

出版信息

Br J Haematol. 1980 Dec;46(4):565-74. doi: 10.1111/j.1365-2141.1980.tb06013.x.

Abstract

Sixty-four patients with monoclonal protein in serum but initially without evidence of multiple myeloma, macroglobulinaemia, amyloidosis or lymphoma, were studied. Fifty patients (78%) were observed for a period of exceeding 3 years. Based on the follow-up data the patients were classified into the following four groups: Group 1 = patients with transient monoclonal gammopathy: 4 . 7%; Group 2 = patients without significant increase in monoclonal serum protein: 75%; Group 3 = patients with more than 50% increase in monoclonal serum protein: 14 . 1%; Group 4 = patients in whom multiple myeloma developed: 6 . 2%. The mean interval from discovery of the serum monoclonal protein to evolution to multiple myeloma was 61 months. Retrospective analysis of age, sex, blood count, bone marrow picture, antigenic type and size of serum monoclonal proteins, presence of small amounts of homogeneous light chain in the urine, serum albumin level, levels of residual immunoglobulins, did not help to distinguish initially the patients in whom the monoclonal gammopathy evolved to multiple myeloma from patients in whom the disease remained benign and stable. The evolution to multiple myeloma had occurred abruptly after long periods of stable condition; and until this progression the follow-up data were similar to the patients with benign disease. The possible physiopathology of occurrence and evolution of benign monoclonal gammopathy is discussed.

摘要

对64例血清中存在单克隆蛋白但最初无多发性骨髓瘤、巨球蛋白血症、淀粉样变性或淋巴瘤证据的患者进行了研究。50例患者(78%)观察期超过3年。根据随访数据,将患者分为以下四组:第1组=短暂性单克隆丙种球蛋白病患者:4.7%;第2组=单克隆血清蛋白无显著增加的患者:75%;第3组=单克隆血清蛋白增加超过50%的患者:14.1%;第4组=发生多发性骨髓瘤的患者:6.2%。从发现血清单克隆蛋白到发展为多发性骨髓瘤的平均间隔时间为61个月。对年龄、性别、血细胞计数、骨髓图像、血清单克隆蛋白的抗原类型和大小、尿中少量均一轻链的存在、血清白蛋白水平、残留免疫球蛋白水平进行回顾性分析,无助于最初区分单克隆丙种球蛋白病发展为多发性骨髓瘤的患者与疾病保持良性和稳定的患者。在长期稳定状态后,多发性骨髓瘤的发展是突然发生的;在这种进展之前,随访数据与良性疾病患者相似。本文讨论了良性单克隆丙种球蛋白病发生和发展的可能病理生理学。

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