PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: We identified 37 native kidney biopsies with LyN and retrospectively obtained clinicopathologic data.
UNASSIGNED: 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.
UNASSIGNED: Increased awareness of the full clinicopathologic spectrum of LyN may lead to prompt diagnosis, earlier treatment, and potentially improved outcome of this rare entity.
UNASSIGNED: We identified 37 native kidney biopsies with LyN and retrospectively obtained clinicopathologic data.
UNASSIGNED: 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.
UNASSIGNED: Increased awareness of the full clinicopathologic spectrum of LyN may lead to prompt diagnosis, earlier treatment, and potentially improved outcome of this rare entity.
摘要：
■溶菌酶相关性肾病（LyN），慢性粒单核细胞白血病（CMML）患者肾损伤的罕见原因，到目前为止还没有得到很好的描述。我们从多机构系列报告了LyN的临床病理谱。
■我们用LyN鉴定了37例天然肾活检，并回顾性获得了临床病理数据。
■37名患者的中位年龄为74岁，其中78％为男性。他们最常见的表现是急性肾损伤（AKI）或慢性肾脏病（CKD）的AKI（66％），中位估计肾小球滤过率（eGFR）为每1.73m221.7ml/min，蛋白尿为1.7g。少数（15％）患有部分范可尼综合征。血清溶菌酶水平在所有测试中均升高。血液系统疾病（n=28，76％）是最常见的病因，包括CMML(n=15)，急性髓系白血病（n=5），骨髓增生异常综合征（MDS）（n=5）。非血液学原因（n=5，14%），包括转移性神经内分泌癌（n=3），结节病,还有麻风病.4例（11%）病因不明。病理显示近端肾小管病伴丰富的嗜酸性粒细胞增多胞浆内包涵体，溶菌酶免疫染色具有特征性的染色模式。死亡率很高（8/30）。然而,在活着的22人中，包括85%的治疗，7有改善肾功能，包括1名停止透析的患者和6名患者,与活检时的eGFR相比,eGFR增加>15ml/min/1.73m2。
■提高对LyN的全部临床病理谱的认识可能会导致及时诊断，早期治疗,并可能改善这种罕见实体的结果。
■我们用LyN鉴定了37例天然肾活检，并回顾性获得了临床病理数据。
■37名患者的中位年龄为74岁，其中78％为男性。他们最常见的表现是急性肾损伤（AKI）或慢性肾脏病（CKD）的AKI（66％），中位估计肾小球滤过率（eGFR）为每1.73m221.7ml/min，蛋白尿为1.7g。少数（15％）患有部分范可尼综合征。血清溶菌酶水平在所有测试中均升高。血液系统疾病（n=28，76％）是最常见的病因，包括CMML(n=15)，急性髓系白血病（n=5），骨髓增生异常综合征（MDS）（n=5）。非血液学原因（n=5，14%），包括转移性神经内分泌癌（n=3），结节病,还有麻风病.4例（11%）病因不明。病理显示近端肾小管病伴丰富的嗜酸性粒细胞增多胞浆内包涵体，溶菌酶免疫染色具有特征性的染色模式。死亡率很高（8/30）。然而,在活着的22人中，包括85%的治疗，7有改善肾功能，包括1名停止透析的患者和6名患者,与活检时的eGFR相比,eGFR增加>15ml/min/1.73m2。
■提高对LyN的全部临床病理谱的认识可能会导致及时诊断，早期治疗,并可能改善这种罕见实体的结果。
