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轻链沉积病:一例形态学病例报告。

Light Chain Deposition Disease: A Morphological Case Report.

作者信息

Popov Hristo, Stoyanov George S, Ghenev Peter

机构信息

General and Clinical Pathology/Forensic Medicine and Deontology, Medical University of Varna, Varna, BGR.

General and Clinical Pathology, St. Marina University Hospital, Varna, BGR.

出版信息

Cureus. 2022 Jun 27;14(6):e26357. doi: 10.7759/cureus.26357. eCollection 2022 Jun.

Abstract

Light chain deposition disease (LCDD) is a rare condition associated with the overproduction and deposition of monoclonal light chain immunoglobulins. The kidneys are universally affected in LCDD, with the development of renal failure with nephrotic syndrome, microscopic hematuria, and proteinuria. Most cases are associated with a plasma cell neoplasm (multiple myeloma or plasmacytoma) or other lymphoproliferative disorders, with a reasonable number of cases also being idiopathic. Other organs can be affected in rare cases, without producing significant symptoms, predominantly the liver and heart. In this report, we discuss a case of a 72-year-old female presenting with the aforementioned symptoms. Percutaneous ultrasound-guided kidney biopsy revealed nodular sclerosis with periodic acid-Schiff stain (PAS)-positive, Congo red-negative, silver impregnation weakly-positive, and Masson's trichrome-positive (red reaction) deposits with ribbon-like changes together with light chain deposits in the tubular basement membranes. As LCDD was diagnosed, the patient was subjected to further tests, with multiple myeloma of the thoracic vertebrae also being diagnosed. The patient was started on myeloma treatment; however, she subsequently developed a severe lower limb infection that required amputation, after which she developed sepsis and expired.

摘要

轻链沉积病(LCDD)是一种罕见疾病,与单克隆轻链免疫球蛋白的过度产生和沉积有关。肾脏在LCDD中普遍受累,会出现伴有肾病综合征、镜下血尿和蛋白尿的肾衰竭。大多数病例与浆细胞瘤(多发性骨髓瘤或浆细胞瘤)或其他淋巴增殖性疾病有关,也有相当数量的病例为特发性。在罕见情况下,其他器官也可能受累,但不产生明显症状,主要是肝脏和心脏。在本报告中,我们讨论了一名72岁女性出现上述症状的病例。经皮超声引导下肾活检显示结节性硬化,高碘酸-希夫染色(PAS)阳性、刚果红阴性、银浸染弱阳性、马松三色染色阳性(红色反应)沉积物伴有带状改变,同时在肾小管基底膜有轻链沉积。确诊为LCDD后,患者接受了进一步检查,同时也诊断出胸椎多发性骨髓瘤。患者开始接受骨髓瘤治疗;然而,她随后发生了严重的下肢感染,需要截肢,之后出现败血症并死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/330b/9326408/77b347906be3/cureus-0014-00000026357-i01.jpg

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