Department of Nephrology, the First Affiliated Hospital of Nanjing Medical University, Nanjing Medical University, Nanjing, China.
Department of Pathology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.
Front Immunol. 2022 Sep 12;13:903315. doi: 10.3389/fimmu.2022.903315. eCollection 2022.
Due to the various clinical and pathological manifestations of kidney involvement in lymphoproliferative disorder (LPD), the whole spectrum of kidney disease in LPD is still unclear, and data on kidney prognosis is scarce.
We retrospectively reviewed the renal pathology profiles from January 2010 to December 2021, and 28 patients with B-cell LPD combined with intact renal biopsy data were included.
There were 20 men and eight women aging 41 to 79 years at the time of renal biopsy (median age 62 years). According to hematological diagnosis, patients were classified into four groups: chronic lymphocytic leukemia (CLL) (group1, n=7), Waldenström macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) (group 2, n=8; WM, n=6; LPL, n=2), Other non-Hodgkin's lymphomas (NHL) (group3, n=7; diffuse large B-cell lymphoma (DLBCL), n=2; mucosa-associated lymphoid tissue (MALT) lymphoma, n=4; Low grade B-cell lymphoma, n=1), and monoclonal gammopathy of undetermined significance/monoclonal gammopathy of renal significance (MGUS/MGRS) (group 4, n=6). Median serum creatinine (Scr) level was 129 (range,59-956) umol/L. Eight patients (29%) were presented with acute kidney injury (AKI), and five patients (18%) required hemodialysis upon admission. Twenty-three patients (82%) presented with proteinuria (median protein excretion, 2.14 g/d), 11(39%) of whom had the nephrotic syndrome. Interstitial malignant infiltration was the most frequent renal lesion (n=6). Eight patients underwent immunohistochemistry of renal tissues, of which three patients (CLL, n=1; LPL, n=1; WM, n=1) had confirmed lymphoma infiltrates, and the infiltrating cells in the remaining five patients (CLL, n=1; MALT lymphoma, n=2; MGUS, n=2) were considered unrelated to lymphoma. The most common glomerular diseases were renal amyloidosis (n=4) and membranous nephropathy (n=4). Only 20 patients were treated, 13 of whom were treated with rituximab separately or in combination. The median follow-up time was 11 months. Of these, six had achieved hematological response, complete response in five cases. Eight had achieved renal response. At the end-of-study visit, four patients died and two progressed to end stage kidney disease (ESKD).
In conclusion, the clinicopathological spectrum of renal involvement in BLPD is diverse. Renal biopsy and immunohistochemistry are required for early diagnosis and prognostic assessment.
由于累及肾脏的淋巴增生性疾病(LPD)具有多种临床和病理学表现,因此 LPD 患者的肾脏疾病全貌仍不清楚,有关肾脏预后的数据也很有限。
我们回顾性分析了 2010 年 1 月至 2021 年 12 月期间的肾脏病理特征,并纳入了 28 例伴有完整肾脏活检数据的 B 细胞 LPD 患者。
28 例患者中,20 例为男性,8 例为女性,在进行肾脏活检时的年龄为 41 岁至 79 岁(中位年龄为 62 岁)。根据血液学诊断,患者分为 4 组:慢性淋巴细胞白血病(CLL)(第 1 组,n=7)、华氏巨球蛋白血症/淋巴浆细胞淋巴瘤(WM/LPL)(第 2 组,n=8;WM,n=6;LPL,n=2)、其他非霍奇金淋巴瘤(NHL)(第 3 组,n=7;弥漫性大 B 细胞淋巴瘤[DLBCL],n=2;黏膜相关淋巴组织[MALT]淋巴瘤,n=4;低级别 B 细胞淋巴瘤,n=1)和单克隆丙种球蛋白病的意义未明/单克隆丙种球蛋白病的意义(MGUS/MGRS)(第 4 组,n=6)。中位血清肌酐(Scr)水平为 129(范围,59-956)μmol/L。8 例患者(29%)表现为急性肾损伤(AKI),5 例患者(18%)入院时需要血液透析。23 例患者(82%)出现蛋白尿(中位蛋白排泄量,2.14 g/d),其中 11 例(39%)患有肾病综合征。间质恶性浸润是最常见的肾脏病变(n=6)。8 例行肾脏组织免疫组化检查,其中 3 例(CLL,n=1;LPL,n=1;WM,n=1)证实有淋巴瘤浸润,其余 5 例(CLL,n=1;MALT 淋巴瘤,n=2;MGUS,n=2)患者的浸润细胞被认为与淋巴瘤无关。最常见的肾小球疾病是肾淀粉样变性(n=4)和膜性肾病(n=4)。仅 20 例患者接受了治疗,其中 13 例单独或联合使用利妥昔单抗。中位随访时间为 11 个月。其中,6 例患者获得血液学缓解,5 例患者完全缓解。8 例患者获得肾脏缓解。在研究结束时,4 例患者死亡,2 例患者进展为终末期肾病(ESKD)。
综上所述,BLPD 患者肾脏受累的临床病理谱多种多样。需要进行肾脏活检和免疫组化检查以进行早期诊断和预后评估。