■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。
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.