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IgA 肾病中轻链沉积的临床病理意义。

Clinicopathological significance of light chain deposition in IgA nephropathy.

机构信息

Kidney Unit, National Hospital Organization, Fukuoka-Higashi Medical Center, Fukuoka, Japan.

Division of Nephrology, Medical Corporation Houshikai, Kano Hospital, 1-2-1, Chuoekimae, Shingu-machi, Kasuya-gun, Fukuoka, 811-0120, Japan.

出版信息

Clin Exp Nephrol. 2021 Jun;25(6):621-632. doi: 10.1007/s10157-021-02026-7. Epub 2021 Mar 1.

Abstract

BACKGROUND

Clinicopathological significance of light chain deposition in IgA nephropathy and the relation of monotypic IgA deposition to bone marrow abnormalities are important issues to be clarified.

METHODS

We retrospectively investigated light chain deposition in 526 patients with IgA nephropathy. We divided the patients into 5 groups according to the balance of intensity of both light chain deposition: lambda monotypic, lambda dominant, polytypic, kappa dominant and kappa monotypic. Clinicopathological parameters were compared among the groups. The relation of monotypic IgA deposition to hematological malignancy was also evaluated.

RESULTS

The prevalence of monotypic IgA deposition was 6.3%, 33 patients (21 lambda and 12 kappa). Thirty-two (4.0%) and 10 patients (1.9%) were classified into lambda and kappa dominant groups, respectively. Polytypic IgA deposition was observed in 455 patients (85.7%). Age of onset, age at biopsy, urinary protein creatinine ratio, the percentage of global glomerulosclerosis, and the degree of IgA and C3 deposition were different among the groups. However, there was no gradual difference according to the groups. No patient with monotypic IgA deposition showed hematological abnormality at biopsy and during follow-up.

CONCLUSIONS

The prevalence of IgA monotypic deposition was extremely low. Clinicopathologically, we could not differentiate patients with monotypic IgA deposition from those with polytypic one and no hematological disorder was documented in patients with monotypic IgA deposition. Whether IgA nephropathy with monotypic IgA deposition and that with polytypic one is the same entity or not, and relation between monotypic IgA deposition and hematological malignancy should be clarified by further investigations.

摘要

背景

IgA 肾病中轻链沉积的临床病理意义以及单克隆 IgA 沉积与骨髓异常的关系是需要阐明的重要问题。

方法

我们回顾性调查了 526 例 IgA 肾病患者的轻链沉积情况。根据轻链沉积强度的平衡,我们将患者分为 5 组:lambda 单克隆、lambda 优势、多克隆、kappa 优势和 kappa 单克隆。比较了各组的临床病理参数。还评估了单克隆 IgA 沉积与血液恶性肿瘤的关系。

结果

单克隆 IgA 沉积的患病率为 6.3%,33 例(21 例 lambda 和 12 例 kappa)。32 例(4.0%)和 10 例(1.9%)分别归入 lambda 和 kappa 优势组。455 例(85.7%)为多克隆 IgA 沉积。发病年龄、活检年龄、尿蛋白肌酐比、全球肾小球硬化百分比以及 IgA 和 C3 沉积程度在各组之间存在差异。然而,没有根据各组出现逐渐差异。没有单克隆 IgA 沉积的患者在活检和随访期间出现血液异常。

结论

IgA 单克隆沉积的患病率极低。临床病理上,我们无法将单克隆 IgA 沉积的患者与多克隆的患者区分开来,并且单克隆 IgA 沉积的患者没有血液系统疾病。是否具有单克隆 IgA 沉积的 IgA 肾病与具有多克隆 IgA 沉积的 IgA 肾病是同一实体,以及单克隆 IgA 沉积与血液恶性肿瘤之间的关系,需要通过进一步的研究来阐明。

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