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酷似急进性肾小球肾炎的肾恶性淋巴瘤。

Malignant lymphoma of the kidney mimicking rapid progressive glomerulonephritis.

作者信息

Weng S-C, Shu K-H, Wen M-C, Cheng C-H, Wu M-J, Yu T-M, Chuang Y-W, Chen C-H

机构信息

Division of Nephrology, Department of Internal Medicine, Tunghai University, Taichung, Taiwan.

出版信息

Clin Nephrol. 2010 Dec;74(6):480-4.

Abstract

Primary renal lymphoma (PRL) is rare and often presents as rapidly progressive renal failure. Most cases of PRL are large-cell lymphomas of B-cell lineage. Herein, we report a 75-year-old female patient with infiltrative CD20 (+) B-cell lymphoma who underwent 4 consecutive courses of chemotherapy with R-CVP (rituximab, cyclophosphamide, vincristine, and prednisone) and after 12 sessions became free from hemodialysis in good general condition. Her serum creatinine level gradually decreased to 4.1 mg/dl with adequate urine output. Unfortunately, a relapse of CD20 (-) lymphoma developed rapidly involving other organs. She died with severe hospital-acquired pneumonia and febrile neutropenia after the last chemotherapy with R-MINE almost 1 year after onset of symptoms. We conclude that renal biopsy enables prompt diagnosis in rapidly progressive renal failure and immunophenotyping and also staging workup of the lymphoma in case of positive biopsy. Though rituximab improved response rate of PRL, it reduced expression of CD20. This may relate to frequent relapse/resistance after rituximab therapy and poor long-term patient survival.

摘要

原发性肾淋巴瘤(PRL)较为罕见,常表现为快速进展的肾衰竭。大多数PRL病例为B细胞系大细胞淋巴瘤。在此,我们报告一例75岁女性浸润性CD20(+)B细胞淋巴瘤患者,该患者接受了4个周期的R-CVP(利妥昔单抗、环磷酰胺、长春新碱和泼尼松)化疗,12次治疗后不再需要血液透析,全身状况良好。她的血清肌酐水平逐渐降至4.1mg/dl,尿量充足。不幸的是,CD20(-)淋巴瘤迅速复发并累及其他器官。在症状出现近1年后,她在接受最后一次R-MINE化疗后死于严重的医院获得性肺炎和发热性中性粒细胞减少症。我们得出结论,肾活检能够在快速进展的肾衰竭中实现快速诊断,进行免疫表型分析,并且在活检呈阳性的情况下对淋巴瘤进行分期检查。虽然利妥昔单抗提高了PRL的缓解率,但它降低了CD20的表达。这可能与利妥昔单抗治疗后频繁复发/耐药以及患者长期生存率低有关。

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