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一名伴有冷球蛋白血症的多发性骨髓瘤患者免疫球蛋白G κ链的异常N-糖基化:病例报告

Abnormal N-glycosylation of the immunoglobulin G kappa chain in a multiple myeloma patient with crystalglobulinemia: case report.

作者信息

Hashimoto Ryo, Toda Tosifusa, Tsutsumi Hisashi, Ohta Masatsugu, Mori Mayumi

机构信息

Department of Hematology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.

出版信息

Int J Hematol. 2007 Apr;85(3):203-6. doi: 10.1532/IJH97.06074.

Abstract

Spontaneous crystallization of monoclonal immunoglobulins (crystalglobulin) is a rare complication of multiple myeloma. We describe a 64-year-old Japanese man with skin ulcers and renal failure associated with immunoglobulin G kappa multiple myeloma. Crystallized immunoglobulin was detected in his serum at room temperature. Analysis of the patient's crystalglobulin by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and mass spectrometry suggested that the crystallization was due to abnormal glycosylation of the immunoglobulin light chain. Treatment with thalidomide and dexamethasone improved the severe skin ulcers on the patient's extremities and partially reversed his renal failure. This report is the first of abnormal glycosylation of immunoglobulin possibly caused by modification of N-glycans in the light chain. We concluded that abnormal glycosylation of the immunoglobulin light chain might be the cause of the patient's skin ulcers and renal dysfunction.

摘要

单克隆免疫球蛋白(冷球蛋白)的自发结晶是多发性骨髓瘤的一种罕见并发症。我们描述了一名64岁的日本男性,患有与免疫球蛋白Gκ多发性骨髓瘤相关的皮肤溃疡和肾衰竭。在室温下于其血清中检测到了结晶的免疫球蛋白。通过十二烷基硫酸钠-聚丙烯酰胺凝胶电泳和质谱对患者的冷球蛋白进行分析表明,结晶是由于免疫球蛋白轻链的异常糖基化所致。沙利度胺和地塞米松治疗改善了患者四肢严重的皮肤溃疡,并部分逆转了他的肾衰竭。本报告首次报道了可能由轻链中N-聚糖修饰引起的免疫球蛋白异常糖基化。我们得出结论,免疫球蛋白轻链的异常糖基化可能是患者皮肤溃疡和肾功能障碍的原因。

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