Department of Pathology, ColumbiaUniversity Medical Center, New York, NY, USA.
Nephrol Dial Transplant. 2013 Mar;28(3):620-31. doi: 10.1093/ndt/gfs537. Epub 2012 Dec 18.
While BK polyoma virus nephropathy (PVN) is a well-recognized cause of renal allograft dysfunction, PVN of native kidneys is likely under-recognized.
We present the pathologic features, risk factors and outcomes of eight cases of PVN in native kidneys.
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.
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.
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。
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。
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。
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。