Christopoulos P D, Katsoudas S, Androulaki A, Nakopoulou L, Economopoulos T, Vlahakos D V
Department of Medicine II, Attikon University Hospital, Medical School of the University of Athens, Athens, Greece.
Clin Nephrol. 2009 Feb;71(2):198-202. doi: 10.5414/cnp71198.
A 65-year-old white female patient with normal baseline renal function was referred to our hospital with nonoliguric renal failure requiring hemodialysis after progressive deterioration over the previous 6 months. Her past medical history was remarkable for easy fatigability, weight loss, low-grade fever, hypogammaglobulinemia and mild hepatosplenomegaly manifested over the past 6 years. Several liver and bone marrow biopsies during that period had shown a nonspecific polyclonal T-cell infiltration, and she was administered low-dose steroids for symptomatic relief. Physical examination, laboratory workup and imaging studies at presentation showed pancytopenia, hepatosplenomegaly, large symmetric kidneys with normal cortices and no evidence of obstructive uropathy, aseptic pyuria with neutrophils and lymphocytes and mild proteinuria. On biopsy the renal interstitium was infiltrated by large, granular CD3+CD8+CD56-CD57+ lymphocytes, clonal by molecular analysis, which established the diagnosis of T-cell large granular lymphocyte leukemia. Most urinary and peripheral blood lymphocytes bore the same T-LGL surface markers and were also clonal, as shown by flow-cytometry and PCR amplification of the T-cell receptor g-chain genes. A subsequent bone marrow biopsy revealed infiltration by lymphoma cells and excluded a myelodysplastic or hemophagocytic syndrome. After exclusion of an underlying EBV, CMV, HBV, HCV or HIV infection with negative serology and blood PCR the patient received one cycle of chemotherapy with cyclophosphamide, vincristine and prednisone. No improvement of renal function was achieved, while complication with a prolonged pulmonary infection and severe sepsis precluded further treatment. Our report indicates that the T-LGL leukemia should be considered in the differential diagnosis of renal failure with large-sized kidneys, especially when hepatosplenomegaly, pancytopenia and aseptic pyuria are also present. In the latter case, flow-cytometric and clonality analysis of the urine sediment can aid in establishing a diagnosis. Since renal function may deteriorate rapidly, chemotherapy should not be delayed.
一名65岁基线肾功能正常的白人女性患者，在过去6个月病情逐渐恶化后，因非少尿型肾衰竭需要血液透析而被转诊至我院。她过去6年有易疲劳、体重减轻、低热、低丙种球蛋白血症及轻度肝脾肿大的病史。在此期间多次肝脏和骨髓活检显示为非特异性多克隆T细胞浸润，她接受了低剂量类固醇治疗以缓解症状。就诊时的体格检查、实验室检查及影像学检查显示全血细胞减少、肝脾肿大、双侧肾脏对称增大且皮质正常、无梗阻性尿路病证据、无菌性脓尿伴中性粒细胞和淋巴细胞及轻度蛋白尿。肾活检显示肾间质被大量颗粒状CD3 + CD8 + CD56 - CD57 +淋巴细胞浸润，分子分析显示为克隆性，从而确诊为T细胞大颗粒淋巴细胞白血病。流式细胞术及T细胞受体γ链基因的PCR扩增显示，多数尿液及外周血淋巴细胞具有相同的T - LGL表面标志物且也是克隆性的。随后的骨髓活检显示有淋巴瘤细胞浸润，排除了骨髓增生异常综合征或噬血细胞综合征。血清学及血液PCR检查排除潜在的EBV、CMV、HBV、HCV或HIV感染后，患者接受了环磷酰胺、长春新碱及泼尼松的一个疗程化疗。肾功能未改善，同时因长期肺部感染及严重脓毒症并发症而无法进一步治疗。我们的报告表明，在鉴别诊断肾脏增大的肾衰竭时应考虑T - LGL白血病，尤其是当同时存在肝脾肿大、全血细胞减少及无菌性脓尿时。在后一种情况下，尿沉渣的流式细胞术及克隆性分析有助于确诊。由于肾功能可能迅速恶化，不应延迟化疗。
