Renal Unit, Department of Medicine, Queen Elizabeth Hospital, Hong Kong.
Division of Nephrology, Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Pok Fu Lam, Hong Kong.
Nephrology (Carlton). 2021 Aug;26(8):650-658. doi: 10.1111/nep.13876. Epub 2021 Mar 23.
Direct kidney involvement in B-cell lymphoproliferative disease is a rare disorder with only a few studies reported in Caucasian patients. The clinicopathological characteristics and outcome of this entity remain poorly described.
We retrospectively studied all adult Chinese patients who had histology-proven renal parenchymal infiltration by malignant B-cells between 1 January 2000 and 31 December 2018 at two tertiary hospitals in Hong Kong. Clinical, pathological and radiological data were collected from 20 patients. Follow-up data were analysed until 31 December 2019.
Median follow-up duration was 22 (1-171) months. Only seven patients (35%) had established diagnosis of haematological cancer before kidney biopsy. Diffuse large B-cell lymphoma (DLBCL) was the most common subtype in our cohort (n = 5, 25%). Others included low-grade B-cell lymphoma (n = 11), intravascular large B-cell lymphoma (n = 1), mantle cell lymphoma (n = 1) and multiple myeloma (n = 2). Fourteen patients (70%) presented with AKI while 12 patients (60%) had proteinuria. Seven patients (35%) had unilateral renal mass, one had bilateral renal masses and one had bilateral diffuse nephromegaly in computed tomography. Lymphomatous tubulointerstitial infiltration was the prevalent histological finding. Nine patients (45%) had coexisting renal lesions other than direct tumour infiltration. All but one patient received chemotherapy. Ten patients died and renal responders had a significantly better survival than non-renal responders (p = .03).
Direct tumour infiltration can occur in a wide variety of B-cell lymphoproliferative disorders. Coexisting immunoglobulin-related nephropathy is frequently found. Renal biopsy is required for early diagnosis which allows timely and appropriate treatment.
B 细胞淋巴增生性疾病直接累及肾脏是一种罕见疾病,仅有少数几项针对白种人群的研究报道。该疾病实体的临床病理特征和结局仍描述不足。
我们回顾性研究了 2000 年 1 月 1 日至 2018 年 12 月 31 日期间在香港两家三级医院接受组织学证实的恶性 B 细胞肾实质浸润的所有成年中国患者。收集了 20 例患者的临床、病理和影像学数据。对随访数据进行了分析,随访时间截至 2019 年 12 月 31 日。
中位随访时间为 22(1-171)个月。仅有 7 例(35%)患者在肾活检前已确诊血液系统恶性肿瘤。弥漫性大 B 细胞淋巴瘤(DLBCL)是本队列中最常见的亚型(n=5,25%)。其他亚型包括低级别 B 细胞淋巴瘤(n=11)、血管内大 B 细胞淋巴瘤(n=1)、套细胞淋巴瘤(n=1)和多发性骨髓瘤(n=2)。14 例(70%)患者表现为急性肾损伤,12 例(60%)患者存在蛋白尿。7 例(35%)患者有单侧肾肿块,1 例患者有双侧肾肿块,1 例患者有双侧弥漫性肾肿大。组织学检查最常见的发现是淋巴瘤性肾小管间质浸润。9 例(45%)患者除直接肿瘤浸润外还有其他并存的肾脏病变。除 1 例患者外,所有患者均接受了化疗。10 例患者死亡,肾脏应答者的生存情况明显优于非肾脏应答者(p=0.03)。
直接肿瘤浸润可发生于多种 B 细胞淋巴增生性疾病。常伴有免疫球蛋白相关的肾脏病变。早期诊断需要进行肾活检,以便及时进行适当的治疗。