利妥昔单抗治疗后缓解的膜性肾病合并系统性轻链淀粉样变性：一例报告
Membranous nephropathy with systemic light-chain amyloidosis of remission after rituximab therapy: A case report.
作者信息
Zhang Jiao, Wang Xu, Zou Gu-Ming, Li Jia-Yi, Li Wen-Ge
机构信息
Beijing University of Chinese Medicine, Beijing 100029, China.
Department of Nephrology, China-Japan Friendship Hospital, Beijing 100029, China.
出版信息
World J Clin Cases. 2023 Aug 16;11(23):5538-5546. doi: 10.12998/wjcc.v11.i23.5538.
BACKGROUND
About 70%-80% of patients with primary membranous nephropathy (MN) have phospholipase A2 receptor (PLA2R) in renal tissue. Systemic light-chain (AL) amyloidosis is the most common type of amyloidosis. MN complicated with amyloidosis is rare.
CASE SUMMARY
A 48-year-old Chinese male presented with nephrotic syndrome, positive serum PLA2R antibody, and positive serum and urine IgG-lambda type M-protein, with a normal ratio of serum-free light-chain level. The patient was diagnosed with MN accompanied by AL amyloidosis. He was treated with rituximab with glucocorticoids and CyBorD regimen of chemotherapy. After 21 mo of follow-up, the patient achieved complete remission regarding nephrotic syndrome without adverse effects of chemotherapy.
CONCLUSION
We report a case of PLA2R-related MN complicated with primary AL amyloidosis only with renal involvement and successfully treated with rituximab, glucocorticoids and chemotherapy.
背景
约70%-80%的原发性膜性肾病（MN）患者肾组织中有磷脂酶A2受体（PLA2R）。系统性轻链（AL）淀粉样变性是最常见的淀粉样变性类型。MN合并淀粉样变性罕见。
病例摘要
一名48岁中国男性，表现为肾病综合征，血清PLA2R抗体阳性，血清及尿液IgG-λ型M蛋白阳性，血清游离轻链水平比值正常。该患者被诊断为MN伴AL淀粉样变性。给予利妥昔单抗联合糖皮质激素及CyBorD化疗方案治疗。随访21个月后，患者肾病综合征完全缓解，无化疗不良反应。
结论
我们报告1例仅累及肾脏的PLA2R相关MN合并原发性AL淀粉样变性病例，经利妥昔单抗、糖皮质激素及化疗成功治疗。
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