溶菌酶相关性肾病的临床病理谱。
Clinicopathologic Spectrum of Lysozyme-Associated Nephropathy.
发表日期：2023 Aug
作者：
Satoru Kudose, L Nicholas Cossey, Pietro A Canetta, Miroslav Sekulic, Christine A Vanbeek, Forest B Huls, Isha Gupta, Lihong Bu, Mariam P Alexander, Lynn D Cornell, Mary E Fidler, Glen S Markowitz, Christopher P Larsen, Vivette D D'Agati, Samih H Nasr, Dominick Santoriello
来源：
Kidney International Reports
摘要：
溶菌酶相关性肾病(LyN)是慢性髓系单核细胞白血病(CMML)患者肾损伤的罕见原因，迄今为止尚未有详细描述。我们报告了多机构系列病例的LyN临床病理谱。我们鉴定了37例LyN的肾脏原位活检，并回顾性获得临床病理数据。37例患者中位年龄为74岁，男性占比78%。他们最常见的表现是急性肾损伤(AKI)或慢性肾脏病(AKI on CKD)(66%)，估计肾小球滤过率(eGFR)中位数为21.7 ml/min/1.73 m2，蛋白尿为1.7 g。少数患者(15%)有部分Fanconi综合征。所有受检者的血清溶菌酶水平升高。血液病(28例，76%)是最常见的病因，包括CMML(15例)、急性髓系白血病(5例)和骨髓增生异常综合征(MDS) (5例)。非血液病病因(5例, 14%)包括转移性神经内分泌癌(3例)、结节病和麻风病。4例病因未明(11%)。病理显示近曲小管病变，大量异染性胞质内涵物，伴有溶菌酶免疫染色的特征性染色。死亡率较高(8/30)。但在22例存活患者中，包括85%接受治疗，7例肾功能改善，包括1例停止透析和6例eGFR增加>15 ml/min/1.73 m2与活检时比较的eGFR。加强对LyN全面临床病理谱的认识可能有助于及时诊断、早期治疗和潜在改善这种罕见疾病的预后。© 2023 International Society of Nephrology. Published by Elsevier Inc.
Lysozyme-associated nephropathy (LyN), a rare cause of kidney injury in patients with chronic myelomonocytic leukemia (CMML), has not been well described to date. We report the clinicopathologic spectrum of LyN from a multi-institutional series.We identified 37 native kidney biopsies with LyN and retrospectively obtained clinicopathologic data.Thirty-seven patients had a median age of 74 years and included 78% males. Their most common presentation was acute kidney injury (AKI) or AKI on chronic kidney disease (CKD) (66%) with median estimated glomerular filtration rate (eGFR) of 21.7 ml/min per 1.73 m2, and proteinuria of 1.7 g. A minority (15%) had partial Fanconi syndrome. Serum lysozyme levels were elevated in all tested. Hematologic disorder (n = 28, 76%) was the most common etiology, including CMML (n = 15), acute myeloid leukemia (n = 5), and myelodysplastic syndrome (MDS) (n = 5). Nonhematologic causes (n = 5, 14%), included metastatic neuroendocrine carcinoma (n = 3), sarcoidosis, and leprosy. Etiology was unknown in 4 (11%). Pathology showed proximal tubulopathy with abundant hypereosinophilic intracytoplasmic inclusions, with characteristic staining pattern by lysozyme immunostain. Mortality was high (8/30). However, among the 22 alive, including 85% treated, 7 had improved kidney function, including 1 who discontinued dialysis and 6 with increase in eGFR >15 ml/min per 1.73 m2 compared with eGFR at the time of biopsy.Increased awareness of the full clinicopathologic spectrum of LyN may lead to prompt diagnosis, earlier treatment, and potentially improved outcome of this rare entity.© 2023 International Society of Nephrology. Published by Elsevier Inc.