• 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Clinical nephrology >T-cell large granular lymphocyte leukemia presenting as end-stage renal disease: the diagnostic role of flow-cytometric and clonality analysis of the urine sediment.
【24h】

T-cell large granular lymphocyte leukemia presenting as end-stage renal disease: the diagnostic role of flow-cytometric and clonality analysis of the urine sediment.

机译：表现为终末期肾脏疾病的T细胞大颗粒淋巴细胞白血病：尿沉渣的流式细胞仪和克隆性分析的诊断作用。

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

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.
机译：一名基线肾功能正常的65岁白人女性患者被转诊至我院，患有非少尿性肾衰竭，在过去6个月进行性恶化后需要进行血液透析。她过去的病史以易疲劳，体重减轻，低烧，低血球蛋白血症和轻度肝脾肿大而著称。在此期间，数次肝和骨髓活检显示非特异性多克隆T细胞浸润，并给予她低剂量类固醇药物以缓解症状。体格检查，实验室检查和影像学检查显示全血细胞减少症，肝脾肿大，大对称肾，皮层正常，无阻塞性尿毒症证据，无菌性脓尿伴中性粒细胞和淋巴细胞，以及轻度蛋白尿。活检时，肾间质被大的，颗粒状的CD3 + CD8 + CD56-CD57 +淋巴细胞浸润，并通过分子分析进行克隆，从而建立了对T细胞大颗粒性淋巴细胞白血病的诊断。如流式细胞术和T细胞受体g链基因的PCR扩增所示，大多数尿液和外周血淋巴细胞带有相同的T-LGL表面标记，并且也是克隆的。随后的骨髓活检显示淋巴瘤细胞浸润，排除骨髓增生异常或噬血细胞综合征。在排除血清学阴性和血液PCR的潜在EBV，CMV，HBV，HCV或HIV感染后，患者接受了一个周期的环磷酰胺，长春新碱和泼尼松化疗。肾功能未见改善，而合并肺部感染延长和严重脓毒症则不能进一步治疗。我们的报告表明，在对大尺寸肾脏进行肾衰竭的鉴别诊断时，应考虑T-LGL白血病，尤其是当还存在肝脾肿大，全血细胞减少和无菌性脓尿时。在后一种情况下，尿沉渣的流式细胞仪和克隆性分析可以帮助建立诊断。由于肾功能可能迅速恶化，因此不应延迟化疗。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号