Department of Nephrology and Renal Replacement, Daido Hospital, 9, Hakusui-cho, Minami-ku, Nagoya, 457-8511, Japan.
Department of Pathology, Daido Hospital, Nagoya, Japan.
CEN Case Rep. 2021 Aug;10(3):320-325. doi: 10.1007/s13730-020-00567-6. Epub 2021 Jan 6.
An 80-year-old man with myelodysplastic syndrome developed acute kidney injury (AKI) and peripheral blood monocyte-dominant leukocytosis. Glomerular disease was suspected from urinalysis, which showed proteinuria and microscopic hematuria with red cell casts. Eventually, he died of respiratory failure, after which a postmortem was performed. In the glomeruli, the extracapillary space was filled with numerous mononuclear cells and erythrocytes. At first interpretation, the glomerular findings appeared to represent cellular crescents. However, immunostaining revealed that the extracapillary mononuclear cells were in fact leukemic cells. Furthermore, tubular injury due to marked accumulation of lysozyme was also recognized together with infiltration of leukemic cells in the interstitium. The diagnosis of kidney infiltration by chronic myelomonocytic leukemia (CMML) and lysozyme-induced tubular injury was eventually made. Our case is the first report showing extracapillary infiltration of leukemic cells by immunostaining. In addition, lysozyme-induced tubular injury is a forgotten cause of kidney injury in patients with CMML. This case teaches us the rare and forgotten causes of AKI with CMML.
一位 80 岁男性患有骨髓增生异常综合征,出现急性肾损伤(AKI)和外周血单核细胞主导性白细胞增多症。尿分析提示肾小球疾病,表现为蛋白尿和镜下血尿伴红细胞管型。最终,他因呼吸衰竭死亡,随后进行了尸检。在肾小球中,毛细血管外间隙充满了大量单核细胞和红细胞。最初的解释是,肾小球的表现似乎代表细胞性新月体。然而,免疫组化显示毛细血管外的单核细胞实际上是白血病细胞。此外,还观察到由于溶菌酶的大量堆积导致的肾小管损伤,以及间质中白血病细胞的浸润。最终诊断为慢性髓单核细胞白血病(CMML)肾脏浸润和溶菌酶诱导的肾小管损伤。本病例是首例通过免疫组化显示毛细血管外浸润白血病细胞的报告。此外,溶菌酶诱导的肾小管损伤是 CMML 患者肾损伤的一个被遗忘的原因。这个病例让我们了解了 CMML 导致 AKI 的罕见和被遗忘的原因。