The Matter of Kidney Biopsy in Monoclonal Gammopathy of Renal Significance: A Case Report of a New Pattern of Immunoglobulin-Storing Histiocytosis.

IF 0.7 Q4 UROLOGY & NEPHROLOGY Case Reports in Nephrology and Dialysis Pub Date : 2023-12-15 eCollection Date: 2023-01-01 DOI:10.1159/000533913

Paolo Randone, Manuel Burdese, Antonella Barreca, Stefania Oliva, Enrico Sanna, Isabella Abbasciano, Patrizia Anania, Elena Boaglio, Luigi Biancone

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Abstract

Monoclonal gammopathy of renal significance (MGRS) represents a group of disorders, characterized by paraproteinemia which causes renal damage. These disorders never meet the diagnostic criteria for multiple myeloma (MM) or lymphoproliferative disease. Crystal-storing histiocytosis is one of the rarest patterns of MGRS, characterized by an accumulation of light chains of crystals within histiocyte's cytoplasm, located in bone marrow or other extramedullary sites such as the kidney, cornea, or thyme. A very few cases have been described as immunoglobulin-storing histiocytosis (IgSH) without evidence of crystals. In the recent literature, only 3 cases of IgSH have been described so far, none renal. In all cases, these very peculiar histopathological patterns are associated with lymphoproliferative or plasma cellular disorders. Here, we report a very unusual IgSH pattern in a kidney biopsy, which led to prompt detection and early therapeutic intervention, in a patient with otherwise misdiagnosed MGRS.

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肾脏单克隆丙种球蛋白病中的肾活检问题：免疫球蛋白蓄积性组织细胞增生症新模式的病例报告。

肾脏单克隆抗体病（MGRS）是一组以引起肾脏损害的副蛋白血症为特征的疾病。这些疾病从来不符合多发性骨髓瘤（MM）或淋巴增生性疾病的诊断标准。晶体贮积性组织细胞增生症是 MGRS 最罕见的类型之一，其特点是组织细胞胞浆内贮积轻链晶体，位于骨髓或其他髓外部位，如肾脏、角膜或甲状腺。极少数病例被描述为免疫球蛋白蓄积性组织细胞增生症（IgSH），但没有晶体的证据。在最近的文献中，迄今只描述了 3 例 IgSH 病例，其中没有一例是肾性的。在所有病例中，这些非常奇特的组织病理学模式都与淋巴增生性疾病或浆细胞疾病有关。在这里，我们报告了肾活检中的一种非常不寻常的 IgSH 模式，这种模式使一名被误诊为 MGRS 的患者得以及时发现并进行早期治疗干预。

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来源期刊

Case Reports in Nephrology and Dialysis UROLOGY & NEPHROLOGY-

CiteScore

1.20

自引率

0.00%

发文量

审稿时长

10 weeks

期刊介绍： This peer-reviewed online-only journal publishes original case reports covering the entire spectrum of nephrology and dialysis, including genetic susceptibility, clinical presentation, diagnosis, treatment or prevention, toxicities of therapy, critical care, supportive care, quality-of-life and survival issues. The journal will also accept case reports dealing with the use of novel technologies, both in the arena of diagnosis and treatment. Supplementary material is welcomed.