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POEMS 综合征:临床实验室从业者角色的重要性。

POEMS syndrome: importance of the clinical laboratory practitioner's role.

机构信息

Department of Veterans Affairs, Robley Rex Medical Center, Louisville, KY 40206, USA.

出版信息

Clin Chim Acta. 2012 Nov 12;413(21-22):1800-7. doi: 10.1016/j.cca.2012.06.032. Epub 2012 Jul 3.

DOI:10.1016/j.cca.2012.06.032
PMID:22771968
Abstract

BACKGROUND

Polyneuropathy organomegaly, endocrinopathy, monoclonal (M) gammopathy, skin syndrome (POEMS) may be difficult to diagnose as a result of methodological and clinical idiosyncrasies. Four of eleven criteria (M-protein, VEGF, endocrinopathy and thrombocytosis/polycythemia) are closely associated with clinical laboratory testing. POEMS has been largely associated with λ-M-gammopathies. Vascular endothelial growth factor (VEGF) is a recent addition to the major diagnostic criteria. VEGF may alter vascular permeability causing some manifestations of POEMS.

METHODS

Review of the literature that focuses on clinical laboratory issues--endocrinological findings, identification of monoclonal gammopathies by electrophoresis and case demonstration.

RESULTS

Based on the criterion of VEGF, POEMS was diagnosed in a patient with a κ-M-gammopathy.

CONCLUSIONS

Low-level IgA monoclonal proteins that are common in POEMS are often difficult to identify by serum electrophoresis (SPE). Immunofixation electrophoresis is required when polyneuropaties are investigated and an M-protein is not identified by SPE. VEGF may improve the sensitivity for diagnosis of POEMS and findings from capillary leak syndrome which are also associated with elevated VEGF and M-gammopathy suggests that κ-M-gammopathies may be implicated more often. It is demonstrated that due to computerized records, the laboratory practitioner is well suited to help the clinician make this complicated diagnosis.

摘要

背景

多发性神经病、器官肿大、内分泌病、单克隆(M)γ球蛋白病、皮肤综合征(POEMS)可能由于方法学和临床特殊性而难以诊断。十一项标准中的四项(M 蛋白、VEGF、内分泌病和血小板增多/红细胞增多症)与临床实验室检测密切相关。POEMS 主要与 λ-M gammopathy 相关。血管内皮生长因子(VEGF)是主要诊断标准中的新增内容。VEGF 可能会改变血管通透性,导致 POEMS 的一些表现。

方法

对重点关注临床实验室问题的文献进行综述,包括内分泌学发现、电泳鉴定单克隆丙种球蛋白和病例展示。

结果

根据 VEGF 标准,诊断了一位患有 κ-M gammopathy 的 POEMS 患者。

结论

POEMS 中常见的低水平 IgA 单克隆蛋白通常很难通过血清电泳(SPE)识别。当研究多发性神经病且 SPE 未识别出 M 蛋白时,需要进行免疫固定电泳。VEGF 可能会提高 POEMS 诊断的敏感性,以及与升高的 VEGF 和 M gammopathy 相关的毛细血管渗漏综合征的发现表明,κ-M gammopathy 可能更为常见。由于计算机记录,实验室医师非常适合帮助临床医生做出这一复杂的诊断。

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