Department of Nephrology, The First Affiliated Hospital of Huzhou Teachers College, the First People's Hospital of Huzhou, Huzhou, 313000, Zhejiang, P.R. China.
Department of Pathology, The First Affiliated Hospital of Huzhou Teachers College, the First People's Hospital of Huzhou, Huzhou, 313000, Zhejiang, P.R. China.
BMC Nephrol. 2023 Oct 6;24(1):297. doi: 10.1186/s12882-023-03351-7.
As a very rare form of B-cell lymphoma, plasmablastic lymphoma (PBL) typically occurs in patients with underlying immunosuppression, including human immunodeficiency virus (HIV), organ transplantation, and autoimmune diseases. For HIV-positive patients, PBL normally originates in the gastrointestinal tract, especially from the oral cavity in most cases. It is extremely rare to find abdominal cavity involvement in PBL, and there has been no previously reported instance of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) attributed to monoclonal IgG (MIgG) lambda secreted by PBL.
We report the case of an HIV-negative female with nephrotic syndrome, renal insufficiency, and multiple swollen lymph nodes. Ascitic fluid cytology revealed a high level of plasmablast-like lymphocytes with the restriction of lambda light chains. Besides, the renal biopsy revealed PGNMID, which could presumably be secondary to MIgG-lambda-secreting by PBL. MIgG-lambda-restricted expression was discovered earlier in the kidney tissue than in the blood.
The diagnostic landscape for PBL is notoriously intricate, necessitating a multifaceted and nuanced approach to mitigate the risks of erroneous identification.
作为一种非常罕见的 B 细胞淋巴瘤，浆母细胞淋巴瘤（PBL）通常发生在有潜在免疫抑制的患者中，包括人类免疫缺陷病毒（HIV）、器官移植和自身免疫性疾病。对于 HIV 阳性患者，PBL 通常起源于胃肠道，大多数情况下来自口腔。在 PBL 中发现腹腔受累极为罕见，以前没有报道过由 PBL 分泌的单克隆免疫球蛋白 M（MIgG）lambda 引起的增殖性肾小球肾炎伴单克隆免疫球蛋白沉积（PGNMID）。
我们报告了一例 HIV 阴性女性肾病综合征、肾功能不全和多个淋巴结肿大的病例。腹水细胞学检查显示浆母细胞样淋巴细胞水平较高，且局限于 lambda 轻链。此外，肾活检显示 PGNMID，可能是由 PBL 分泌的 MIgG-lambda 引起的。在肾脏组织中发现 MIgG-lambda 受限表达的时间早于血液。
PBL 的诊断情况非常复杂，需要采用多方面和细致的方法来降低错误识别的风险。
