单克隆免疫球蛋白作为副蛋白血母细胞增多症和瓦尔登斯特伦巨球蛋白血症骨损伤严重程度的预后因素

O. Pisarevskaya, S. A. Alekseev, O. Rukavitsyn
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The first group (0) included patients who did not have osteodestructive changes in the bones. In patients of the second group, a mild degree (I) osteodestructive process was observed, and in patients from the third and fourth groups – moderate (II) and severe (III) degrees, respectively. All patients underwent protein electrophoresis followed by immunofixation to determine the type of paraprotein and its concentration in serum and urine.Results. In the majority of patients, paraproteins were detected in the blood – Gκ (35.1 %), Gλ (24.6 %), Bence Jones protein λ-type (BJλ) (14.9 %); in urine – BJλ protein (14.9 %) and Bence Jones protein κ-type (BJκ) (28.1 %). Secretion of other types of paraproteins in the blood was less frequently detected – Aκ (9.6 %), Aλ (7.0 %), Mκ (3.5 %), Mλ (3.5 %), Dλ (2.6 %), BJκ (4.4 %). 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引用次数: 0

摘要

目的确定发生骨破坏综合征的危险因素。确定副蛋白血母细胞增多症(PH)和瓦尔登斯特伦巨球蛋白血症(WM)患者分泌的单克隆免疫球蛋白(副蛋白)类型与骨破坏综合征严重程度之间的关系。对 116 名 PH 和 WM 患者的数据进行了回顾性分析。104名患者(89.6%)被诊断为多发性骨髓瘤。较少见的是WM(8例患者,占6.9%)、浆细胞白血病(2例患者,占1.8%)、单浆细胞瘤和意义不明的单克隆丙种球蛋白病各1例(占0.9%)。根据骨破坏综合征的严重程度,所有患者被分为 4 组。第一组(0)包括骨骼没有发生骨质破坏性改变的患者。第二组患者的骨破坏过程为轻度(I),第三组和第四组患者的骨破坏过程分别为中度(II)和重度(III)。所有患者均接受了蛋白质电泳和免疫固定检查,以确定副蛋白质的类型及其在血清和尿液中的浓度。大多数患者的血液中检测到副蛋白--Gκ(35.1%)、Gλ(24.6%)、本斯-琼斯蛋白λ型(BJλ)(14.9%);尿液中检测到副蛋白--BJλ蛋白(14.9%)和本斯-琼斯蛋白κ型(BJκ)(28.1%)。血液中其他类型副蛋白的分泌较少:Aκ(9.6 %)、Aλ(7.0 %)、Mκ(3.5 %)、Mλ(3.5 %)、Dλ(2.6 %)、BJκ(4.4 %)。43(37.1%)和 40(34.5%)名患者被诊断为 I 级和 II 级严重骨质破坏综合征;20(17.2%)名患者被诊断为 III 级溶解性破坏,13(11.2%)名患者未被诊断为骨质破坏(0 级)。值得注意的是,多发性骨髓瘤患者血液中出现副蛋白血症 Dλ 和 BJλ 以及高钙血症时,骨质破坏程度(II、III 度)较高。分泌单克隆蛋白 Ak 和 Mλ 的患者被诊断为最低程度(0,I)的骨质破坏综合征。血液中副蛋白Gκ、Gλ、Aλ、Mκ、BJκ以及尿液中蛋白质BJκ和BJλ的分泌类型与骨质破坏过程的严重程度之间没有统计学意义上的显著关系。研究结果有助于确定危险人群,在评估 PH 和 WM 患者骨破坏综合征的严重程度时,可将副蛋白类型、血清钙浓度等参数视为预后因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Monoclonal immunoglobulin as a prognostic factor for the severity of bone damage in paraproteinemic hemoblastoses and Waldenström’s macroglobulinemia
Aim. Identify risk factors for the development of osteodestructive syndrome. To determine the relationship between the types of secreted monoclonal immunoglobulin (paraprotein) and the severity of osteodestructive syndrome in patients with paraproteinemic hemoblastoses (PH) and Waldenström’s macroglobulinemia (WM).Materials and methods. A retrospective analysis of data from 116 patients with PH and WM was performed. 104 patients (89.6 %) were diagnosed with multiple myeloma. Less commonly observed were WM (in 8 patients – 6.9 %), plasma cell leukemia (in 2 patients – 1.8 %), solitary plasmacytoma and monoclonal gammopathy of unknown significance were diagnosed in one case (0.9 %) each. According to the severity of osteodestructive syndrome, all patients were divided into 4 groups. The first group (0) included patients who did not have osteodestructive changes in the bones. In patients of the second group, a mild degree (I) osteodestructive process was observed, and in patients from the third and fourth groups – moderate (II) and severe (III) degrees, respectively. All patients underwent protein electrophoresis followed by immunofixation to determine the type of paraprotein and its concentration in serum and urine.Results. In the majority of patients, paraproteins were detected in the blood – Gκ (35.1 %), Gλ (24.6 %), Bence Jones protein λ-type (BJλ) (14.9 %); in urine – BJλ protein (14.9 %) and Bence Jones protein κ-type (BJκ) (28.1 %). Secretion of other types of paraproteins in the blood was less frequently detected – Aκ (9.6 %), Aλ (7.0 %), Mκ (3.5 %), Mλ (3.5 %), Dλ (2.6 %), BJκ (4.4 %). Osteodestructive syndrome of I and II severity was diagnosed in 43 (37.1 %) and 40 (34.5 %) patients, respectively; lytic destruction of III degree was less frequently detected in 20 (17.2 %) patients, in 13 (11.2 %) patients osteodestruction was not detected (degree 0). It was noted that a higher degree of destruction (II, III) was observed in patients with multiple myeloma occurring with paraproteinemia Dλ and BJλ in the blood, as well as hypercalcemia. Osteodestructive syndrome of the lowest degree (0, I) was diagnosed in patients with the secretion of monoclonal proteins Ak and Mλ. There was no statistically significant relationship between the type of secretion of paraproteins Gκ, Gλ, Aλ, Mκ, BJκ in the blood, as well as proteins BJκ and BJλ in the urine and the severity of the osteodestructive process.Conclusion. The results obtained in the study make it possible to identify risk groups, and parameters such as the type of paraprotein, the concentration of calcium in the blood serum can be considered as prognostic factors when assessing the severity of osteodestructive syndrome in patients with PH and WM.
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