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BK 多瘤病毒肾病,发生于原肾。

BK polyoma virus nephropathy in the native kidney.

机构信息

Department of Pathology, ColumbiaUniversity Medical Center, New York, NY, USA.

出版信息

Nephrol Dial Transplant. 2013 Mar;28(3):620-31. doi: 10.1093/ndt/gfs537. Epub 2012 Dec 18.

Abstract

BACKGROUND

While BK polyoma virus nephropathy (PVN) is a well-recognized cause of renal allograft dysfunction, PVN of native kidneys is likely under-recognized.

METHODS

We present the pathologic features, risk factors and outcomes of eight cases of PVN in native kidneys.

RESULTS

The cohort included eight males aged 16-73 years (mean 47.4) with an immunocompromised state (mean duration 3.15 years) attributable to: hematologic malignancies (n = 6), for which three had undergone bone marrow transplant; lung transplant (n = 1) and combined tuberculosis and diabetes (n = 1). Seven patients were receiving specific immunosuppressive therapies. Patients were biopsied for acute kidney injury (AKI) with rise in mean creatinine levels from baseline 1.6 to 2.8 mg/dL. Pathology showed BK PVN with characteristic intranuclear inclusions staining positive for SV40 T antigen and negative for JC virus (JCV), with positive serum and/or urine PCR for BK virus. One patient had focal medullary JCV co-infection. Two patients also had renal infiltration by chronic lymphocytic leukemia (CLL). Six patients received specific therapy directed to PVN (cidofovir or leflunomide). Follow-up ranged from 2 to 20 (mean 10) months. Despite marked decrease in serum BK viral copy numbers, creatinine continued to rise in six cases (mean 3.7 mg/dL in four, requiring dialysis in two) and three patients died of malignancy, opportunistic infection or renal failure. Advanced histologic stage of PVN, ineffective antiviral therapy, co-morbidities and persistent immunocompromised state likely contributed to the poor outcomes.

CONCLUSION

A high level of suspicion in immunocompromised patients is needed to diagnose PVN in an early stage that may respond more favorably to antiviral therapy.

摘要

背景

BK 多瘤病毒肾病(PVN)是肾移植功能障碍的一个公认病因,但人们可能对原发性肾脏中的 PVN 认识不足。

方法

我们介绍了 8 例原发性肾脏 PVN 的病理特征、危险因素和结局。

结果

该队列包括 8 例男性,年龄 16-73 岁(平均 47.4 岁),免疫功能低下状态的病因包括:血液恶性肿瘤(n=6),其中 3 例接受了骨髓移植;肺移植(n=1)和结核合并糖尿病(n=1)。7 例患者正在接受特定的免疫抑制治疗。患者因急性肾损伤(AKI)接受活检,基线时的平均肌酐水平从 1.6mg/dL 升高至 2.8mg/dL。病理显示具有特征性核内包涵体的 BK PVN,SV40 T 抗原染色阳性,JC 病毒(JCV)染色阴性,BK 病毒血清和/或尿液 PCR 阳性。1 例患者存在局灶性髓质 JCV 合并感染。2 例患者还存在慢性淋巴细胞白血病(CLL)肾浸润。6 例患者接受了针对 PVN 的特定治疗(更昔洛韦或来氟米特)。随访时间从 2 至 20 个月(平均 10 个月)。尽管血清 BK 病毒拷贝数显著下降,但 6 例患者的肌酐持续升高(4 例的肌酐平均升高 3.7mg/dL,2 例需要透析),3 例患者因恶性肿瘤、机会性感染或肾衰竭而死亡。PVN 的晚期组织学阶段、抗病毒治疗无效、合并症和持续的免疫抑制状态可能导致了不良结局。

结论

在免疫功能低下的患者中,需要高度怀疑 PVN,以便在早期阶段进行诊断,从而更有利于接受抗病毒治疗。

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