Gnanasampanthan Sahana, Samelis Vasileios P, Roufosse Candice, Kousios Andreas
Renal Medicine, Royal London Hospital, Barts Health NHS Trust, London, GBR.
Medicine, School of Medicine, European University of Cyprus, Nicosia, CYP.
Cureus. 2025 Jun 10;17(6):e85740. doi: 10.7759/cureus.85740. eCollection 2025 Jun.
Monoclonal immunoglobulin deposition disease (MIDD) is a complication of plasma cell dyscrasias, resulting in abnormal immunoglobulin deposition along basement membranes. We describe a case of a 60-year-old male with a complex hospital admission, presenting with critical illness accompanied by acute kidney injury, nephrotic syndrome and moderately elevated serum free light chain (SFLC) ratio, on a background of well-controlled diabetes, hypertension and chronic kidney disease. There was no clear aetiology for his presentation following preliminary examination and investigations, which led to a biopsy diagnosis of heavy chain deposition disease (HCDD) in the context of monoclonal gammopathy of renal significance (MGRS). We explore the importance of understanding the disease course to allow timely biopsy diagnosis and treatment initiation. Our patient required very close follow-up and a wide multi-disciplinary approach, including haematologists, nephrologists and histopathologists, to guide management in a disease for which therapeutic strategies are poorly defined due to limited clinical trial data.
单克隆免疫球蛋白沉积病(MIDD)是浆细胞异常增生的一种并发症,可导致免疫球蛋白沿基底膜异常沉积。我们描述了一例60岁男性复杂的住院病例,该患者以危重症入院,伴有急性肾损伤、肾病综合征以及血清游离轻链(SFLC)比值中度升高,其基础疾病为控制良好的糖尿病、高血压和慢性肾脏病。初步检查和调查后,其病情的病因尚不明确,最终在具有肾脏意义的单克隆丙种球蛋白病(MGRS)背景下,经活检诊断为重链沉积病(HCDD)。我们探讨了了解疾病病程对于及时进行活检诊断和开始治疗的重要性。我们的患者需要密切随访,并采用多学科综合方法,包括血液科医生、肾内科医生和组织病理学家,以指导对这种因临床试验数据有限而治疗策略尚不明确的疾病的管理。