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肿瘤性单克隆丙种球蛋白病中的血清游离轻链:与κ轻链相比,肿瘤性单克隆丙种球蛋白病患者中λ显性κ/λ比值相对检测不足,且游离λ轻链产生不足。

Serum Free Light Chains in Neoplastic Monoclonal Gammopathies: Relative Under-Detection of Lambda Dominant Kappa/Lambda Ratio, and Underproduction of Free Lambda Light Chains, as Compared to Kappa Light Chains, in Patients With Neoplastic Monoclonal Gammopathies.

作者信息

Lee Won Sok, Singh Gurmukh

机构信息

Medical College of Georgia at Augusta University, Augusta, GA 30912, USA.

出版信息

J Clin Med Res. 2018 Jul;10(7):562-569. doi: 10.14740/jocmr3383w. Epub 2018 Jun 4.

DOI:10.14740/jocmr3383w
PMID:29904440
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5997416/
Abstract

BACKGROUND

Quantitative evaluation of serum free light chains is recommended for the work up of monoclonal gammopathies. Immunoglobulin light chains are generally produced in excess of heavy chains. In patients with monoclonal gammopathy, κ/λ ratio is abnormal less frequently with lambda chain lesions. This study was undertaken to ascertain if the levels of overproduction of the two light chain types and their detection rates are different in patients with neoplastic monoclonal gammopathies.

METHODS

Results of serum protein electrophoresis (SPEP), serum protein immunofixation electrophoresis (SIFE), urine protein electrophoresis (UPEP), urine protein immunofixation electrophoresis (UIFE), and serum free light chain assay (SFLCA) in patients with monoclonal gammopathies were examined retrospectively.

RESULTS

The κ/λ ratios were appropriately abnormal more often in kappa chain lesions. Ratios of κ/λ were normal in about 25% of patients with lambda chain lesions in whom free homogenous lambda light chains were detectable in urine. An illustrative case suggests underproduction of free lambda light chains, in some instances.

CONCLUSIONS

The lower prevalence of lambda dominant κ/λ ratio in lesions with lambda light chains is estimated to be due to relative under-detection of lambda dominant κ/λ ratio in about 25% of the patients and because lambda chains are not produced in as much excess of heavy chains as are kappa chains, in about 5% of the patients. The results question the medical necessity and clinical usefulness of the serum free light chain assay. UPEP/UIFE is under-utilized.

摘要

背景

对于单克隆丙种球蛋白病的检查,建议对血清游离轻链进行定量评估。免疫球蛋白轻链的产生通常超过重链。在单克隆丙种球蛋白病患者中,λ链病变时κ/λ比值异常的情况较少见。本研究旨在确定肿瘤性单克隆丙种球蛋白病患者中两种轻链类型的过量产生水平及其检测率是否存在差异。

方法

回顾性检查单克隆丙种球蛋白病患者的血清蛋白电泳(SPEP)、血清蛋白免疫固定电泳(SIFE)、尿蛋白电泳(UPEP)、尿蛋白免疫固定电泳(UIFE)和血清游离轻链测定(SFLCA)结果。

结果

κ链病变时κ/λ比值异常更为常见。在约25%尿中可检测到游离均一λ轻链的λ链病变患者中,κ/λ比值正常。一个典型病例提示在某些情况下存在游离λ轻链产生不足。

结论

λ轻链病变中λ为主的κ/λ比值患病率较低,估计是由于约25%的患者中λ为主的κ/λ比值检测相对不足,以及约5%的患者中λ链产生比重链过量的程度不如κ链。这些结果对血清游离轻链测定的医学必要性和临床实用性提出了质疑。UPEP/UIFE未得到充分利用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/588f/5997416/3033255d16b2/jocmr-10-562-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/588f/5997416/3033255d16b2/jocmr-10-562-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/588f/5997416/3033255d16b2/jocmr-10-562-g001.jpg

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