Characteristics of Renal Intravascular Large B-cell Lymphoma.
Characteristics of Renal Intravascular Large B-cell Lymphoma.
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DOI：
10.1016/j.ekir.2022.12.001
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发表时间：
2023-03
影响因子：
6
通讯作者：
Dai, Dao-Fu
中科院分区：
文献类型：
--
作者：
Rastogi, Prerna;Alrwashdeh, Audai;Caza, Tiffany;Lin, Mercury;Obeidat, Mohammad;Giannini, Gabriel;Larsen, Chris;Dai, Dao-Fu
肾血管内大B细胞淋巴瘤(IVLBCL)是一种罕见的侵袭性B细胞淋巴瘤，肿瘤细胞占据血管管腔，迄今仅有53例报道。在这里，我们提出了最大的病例系列来描述这种罕见的疾病。我们对诊断为肾脏IVLBCL的肾脏活检和尸检进行了多机构的回顾性回顾，并报告了我们的发现。我们在肾活检中发现20例IVLBCL患者，平均年龄65.7±7.8岁(男性占55%)。最常见的临床表现是发热和贫血。急性肾损伤(AKI)发生率为70%~90%，蛋白尿发生率为70%~84.1%，血尿发生率为45%，肾病范围蛋白尿发生率为10%~26.1%。血清肌酐中位数(四分位数范围)为1.75(1.14，3.3)mg/dl。肿瘤淋巴样细胞存在于肾小球、肾小管周围毛细血管、动脉或静脉。肾外侵犯骨髓、肝、脾、中枢鞭毛系统、肺和皮肤占44.3%。肿瘤细胞表达CD20、CD79a、PAX-5和MUM1+，CD10阴性。可获得的随访数据显示，确诊后中位生存期为21个月。肾外受累是死亡率的重要独立预测因子，在控制了年龄和性别后，危险比为4.975(95%可信区间：1.38，17.88)。血清肌酐、年龄、性别和肾内动脉或静脉的浸润度对存活率没有影响。肾脏IVLBCL是一种罕见的疾病，通过肾脏活检意外诊断，表现为发热、贫血、轻度AKI和蛋白尿。中位生存期为21个月，肾外受累与较差的预后相关。
Renal intravascular large B-cell lymphoma (IVLBCL) is a rare, aggressive B-cell lymphoma with neoplastic cells occupying the vascular lumina with only 53 patients reported to date. Here, we present the largest case series to characterize this rare disease. We performed a multi-institutional, retrospective review of kidney biopsies and autopsies with a diagnosis of kidney IVLBCL and report our findings. We identified 20 patients with an average age of 65.7 ± 7.8 years (55% males) with IVLBCL on kidney biopsy. The most common clinical presentation was fever and anemia. Acute kidney injury (AKI) was noted in 70% to 90%, proteinuria in 70% to 84.1%, hematuria in 45%, and nephrotic-range proteinuria in 10% to 26.1% of cases. The median (interquartile range) of serum creatinine was 1.75 (1.14, 3.3) mg/dl. Neoplastic lymphoid cells were present in glomeruli, peritubular capillaries, and arteries or veins. Of the patients, 44.3% showed extrarenal infiltration into bone marrow, liver, spleen, central vervous system, lung and skin. Neoplastic cells express CD20, CD79a, PAX-5, and MUM1+, and were CD10-negative. Available follow-up data showed a median survival of 21 months after diagnosis. Extrarenal involvement is a significant and independent predictor of mortality with a hazard ratio of 4.975 (95% confidence interval:1.38, 17.88) after controlling for age and gender. Serum creatinine, age, sex, and infiltration of intrarenal arteries or veins did not affect survival. Kidney IVLBCL is a rare disease that is unexpectedly diagnosed by kidney biopsy, presenting with fever, anemia, mild AKI, and proteinuria. Median survival is 21 months and extrarenal involvement is associated with worse outcome.
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影响因子：
3.4
作者：
Hakroush, Samy;Lehnig, Luca-Yves;Koziolek, Michael Johann
通讯作者：
Koziolek, Michael Johann
影响因子：
4.6
作者：
Deisch, Jeremy;Fuda, Franklin 'Buddy';Wang, Huan-You
通讯作者：
Wang, Huan-You
影响因子：
5.7
作者：
Shimada, Kazuyuki;Murase, Takuhei;Kinoshita, Tomohiro
通讯作者：
Kinoshita, Tomohiro
影响因子：
5.5
作者：
Ong YC;Kao HW;Chuang WY;Hung YS;Lin TL;Chang H;Kuo MC
通讯作者：
Kuo MC
影响因子：
5.7
作者：
Shimada K;Kiyoi H
通讯作者：
Kiyoi H