Shimada Satoshi, Nakamichi Takashi, Yamada Gen, Narumi Kaori, Usubuchi Hajime, Yamamoto Tae, Ichikawa Satoshi, Fukuhara Noriko, Miyazaki Mariko, Harigae Hideo, Sato Hiroshi, Ito Sadayoshi
Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
Department of Pathology, Tohoku University Hospital, Sendai, Japan.
CEN Case Rep. 2018 Nov;7(2):248-252. doi: 10.1007/s13730-018-0336-z. Epub 2018 May 15.
A recent systematic review showed that hematological malignancy is often complicated by membranous nephropathy (MN). Histologically, the deposition of IgG subclasses other than IgG4 may imply secondary MN, such as malignancy-associated MN (M-MN). We describe a very rare case of concurrent isolated IgG2-positive MN and B-cell lymphoma. An 83-year-old woman was hospitalized at our institute for facial and lower extremity edema persisting for 2 months. Laboratory tests showed urinary protein level of 10.8 g/day, serum albumin level of 1.6 g/dl, and serum creatinine level of 2.34 mg/dl. Soon after diagnosis of nephrotic syndrome, treatment with corticosteroid was initiated, but it proved to be ineffective. Renal biopsy showed isolated IgG2-positive MN with highly infiltrated CD20-positive lymphoid cells in the kidney. Computed tomography revealed systemic lymphadenopathy, and aberrant B-cells with immunoglobulin light chain restriction were detected in peripheral blood and bone marrow, which led to the diagnosis of mature B-cell lymphoma. Although rituximab (375 mg/m/week) was administered, the patient suddenly died from gastrointestinal bleeding on day 40 of hospitalization. It is, thus, necessary to consider hematological malignancy when a diagnosis of MN is made. Further studies are expected to elucidate the pathogenesis and to help establish the adequate treatment for this rare situation.
最近的一项系统评价显示,血液系统恶性肿瘤常并发膜性肾病(MN)。从组织学上看,除IgG4外的IgG亚类沉积可能提示继发性MN,如恶性肿瘤相关MN(M-MN)。我们描述了一例非常罕见的同时发生孤立性IgG2阳性MN和B细胞淋巴瘤的病例。一名83岁女性因面部和下肢水肿持续2个月入住我院。实验室检查显示尿蛋白水平为10.8g/天,血清白蛋白水平为1.6g/dl,血清肌酐水平为2.34mg/dl。肾病综合征诊断后不久,开始使用皮质类固醇治疗,但证明无效。肾活检显示孤立性IgG2阳性MN,肾脏中有高度浸润的CD20阳性淋巴细胞。计算机断层扫描显示全身淋巴结肿大,外周血和骨髓中检测到具有免疫球蛋白轻链限制的异常B细胞,从而诊断为成熟B细胞淋巴瘤。尽管给予了利妥昔单抗(375mg/m²/周),但患者在住院第40天突然死于胃肠道出血。因此,在诊断MN时需要考虑血液系统恶性肿瘤。期望进一步的研究能够阐明发病机制,并有助于为这种罕见情况制定适当的治疗方案。