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一名多发性骨髓瘤患者出现聚合性(推测为四聚体)λ本-周蛋白血症但无蛋白尿。

Polymeric (presumed tetrameric) lambda Bence Jones proteinemia without proteinuria in a patient with multiple myeloma.

作者信息

Hom B L

出版信息

Am J Clin Pathol. 1984 Nov;82(5):627-9.

PMID:6437210
Abstract

A 55-year-old man presented with nerve compression and examination of tissue removed by laminectomy, and bone marrow aspiration was diagnostic of multiple myeloma. Protein studies showed a total serum protein of 5.7 g/dL, with a M-component in the fast beta region. The abnormal protein reacted only with anti-lambda antisera but not with antisera from multiple sources in four different laboratories, against known heavy chain and kappa-chain determinants. A marked difference in potency of commercial anti-lambda antisera was noted. The reaction was primarily demonstrable in serum and present only in 100-times concentrated urine. Gel filtration disclosed a molecular weight of 84,000. The patient has been followed for the past four years and has not demonstrated significant proteinuria. The English and Japanese literature records seven cases of tetrameric Bence Jones multiple myeloma or plasma cell dyscrasia. This case appears to be the eighth recorded cases of tetrameric Bence Jones proteinemia, the fifth case without proteinuria, and the fifth case involving lambda light chains.

摘要

一名55岁男性因神经受压就诊,经椎板切除术切除组织检查及骨髓穿刺确诊为多发性骨髓瘤。蛋白质研究显示血清总蛋白为5.7 g/dL,在快速β区有一个M成分。异常蛋白仅与抗λ抗血清反应,而在四个不同实验室中,与针对已知重链和κ链决定簇的多种来源抗血清均无反应。注意到市售抗λ抗血清的效价存在显著差异。该反应主要在血清中可检测到,且仅存在于浓缩100倍的尿液中。凝胶过滤显示分子量为84,000。该患者在过去四年中一直接受随访,未出现明显蛋白尿。英文和日文文献记载了7例四聚体本-周蛋白多发性骨髓瘤或浆细胞发育异常病例。该病例似乎是有记录的第8例四聚体本-周蛋白血症病例,第5例无蛋白尿病例,以及第5例涉及λ轻链的病例。

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