Takahashi Haruka, Sano Takashi, Kawamura Sayumi, Sano Keiko, Miyasaka Ryoma, Yamazaki Takuya, Sakakibara Mayuko, Abe Tetsuya, Hashimoto Keiko, Nagaoka Miki, Kamata Mariko, Naito Shokichi, Aoyama Togo, Moriya Rika, Takeuchi Yasuo
Department of Nephrology, Kitasato University School of Medicine, Sagamihara, Japan.
CEN Case Rep. 2022 May;11(2):184-190. doi: 10.1007/s13730-021-00648-0. Epub 2021 Sep 26.
We report a case of immunotactoid glomerulopathy (ITG) complicated with diffuse large B-cell lymphoma (DLBCL). A 68-year-old woman presented with leg edema and was diagnosed with nephrotic syndrome (NS). Renal biopsy revealed ITG. We treated the patient with prednisolone (20 mg/day) and she achieved complete remission of NS. Steroids were gradually reduced. After 1 year, the patient presented with a breast mass determined on biopsy to be DLBCL. She underwent six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) therapy. Follow-up revealed complete remission of both DLBCL and ITG. NS recurred after 5 years and she was simultaneously diagnosed with recurrence of DLBCL in bone marrow. She underwent four cycles of R-EPOCH (rituximab, etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin) therapy and entered remission for DLBCL. NS improved, but the treatment did not lead to remission. After 2 additional years, NS and DLBCL recurred again. She was administered rituximab and NS improved, although proteinuria tended to increase thereafter. One year later, we started prednisolone (10 mg/day), and proteinuria tended to decrease. She is currently undergoing outpatient follow-up. This case suggests that ITG with MGUS should be treated with the possibility of developing malignant hematological disease during the course.
我们报告一例免疫触须样肾小球病(ITG)合并弥漫性大B细胞淋巴瘤(DLBCL)的病例。一名68岁女性因腿部水肿就诊,被诊断为肾病综合征(NS)。肾活检显示为ITG。我们用泼尼松龙(20毫克/天)治疗该患者,她的NS完全缓解。逐渐减少类固醇剂量。1年后,患者出现乳腺肿块,活检确定为DLBCL。她接受了六个周期的R-CHOP(利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松龙)治疗。随访显示DLBCL和ITG均完全缓解。5年后NS复发,同时她被诊断为骨髓中DLBCL复发。她接受了四个周期的R-EPOCH(利妥昔单抗、依托泊苷、泼尼松龙、长春新碱、环磷酰胺、阿霉素)治疗,DLBCL进入缓解期。NS有所改善,但治疗未导致缓解。又过了2年,NS和DLBCL再次复发。给予她利妥昔单抗治疗后NS有所改善,尽管此后蛋白尿有增加趋势。1年后,我们开始使用泼尼松龙(10毫克/天),蛋白尿有减少趋势。她目前正在门诊随访。该病例提示,伴有意义未明的单克隆丙种球蛋白病(MGUS)的ITG在病程中应考虑有发生恶性血液病的可能性并进行相应治疗。