目的：通过分析1例伴双克隆副蛋白血症的轻重链型肾淀粉样变患者诊疗情况，结合文献复习，探讨非传统免疫球蛋白相关肾淀粉样变的诊疗思路。 方法：收集和回顾1例在上海交通大学医学院附属瑞金医院肾脏科诊断为双克隆副蛋白血症的肾淀粉样变患者2021年至2024年间长期诊治随访的临床资料，并复习国内外相关文献。 结果：72岁男性，以泡沫尿、颜面及双下肢水肿为主要症状，血清中检出M蛋白为IgM-λ型和IgA-λ型，肾活检为轻重链型肾淀粉样变（IgA-λ），心脏无明显受累，骨髓中致病克隆为CD38⁺的B淋巴细胞，骨髓病理未检出MYD88基因L265P突变，无淋巴结肿大或结外病灶累及，考虑血液基础疾病为B淋巴细胞增殖性疾病。在前期利妥昔单抗为基础的方案治疗后，调整为靶向CD38的达雷妥尤单抗联合来那度胺，患者快速获得血液学完全缓解和肾脏反应，并在后续治疗中达到肾脏完全缓解。文献复习显示，双克隆副蛋白血症伴肾淀粉样变见于少数个案报道，伴轻重链型肾淀粉样变仅1例。B细胞或淋巴浆细胞克隆致病的淀粉样变中，基于利妥昔单抗的方案是主要治疗手段，但血液学和器官反应均不理想，达雷妥尤单抗联合用药方案可能对CD38⁺的克隆性B细胞致病者有效。 结论：目前双克隆副蛋白淀粉样变尚无诊疗共识或指南，准确判断致病性单克隆细胞、确定治疗靶点、制定个体化联合用药方案，有助于快速获得血液和器官的更深度缓解。

Objective To explore the diagnostic and therapeutic strategies for non-traditional immunoglobulin-related renal amyloidosis by analyzing the clinical management of a patient with heavy and light chain renal amyloidosis and biclonal paraproteinemia. Methods The clinical data of a patient diagnosed with biclonal paraproteinemia and renal amyloidosis at the Department of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine and long-term follow-up from 2021 to 2024 were collected and analyzed, and relevant domestic and foreign literature was reviewed. Results The main symptoms in a 72-year-old male presented with foamy urine, facial and bilateral lower limb edema. Biclonal (IgM κ and IgA, λ) gammopathy were detected, heavy and light-chain renal amyloidosis (IgA-λ) was diagnosed by renal biopsy. There was no obvious involvement in the heart. A small number of monoclonal CD38⁺ B cells were detected through immunophenotyping in bone marrow, while the L265P mutation of the MYD88 gene was negative in it. There was no lymph node enlargement or extranodal lesions, the underlying hematological disease was a B-lymphocyte proliferative disorder. After initial treatment with a rituximab-based regimen, the treatment was adjusted to daratumumab combined with lenalidomide which was targeting CD38. The patient quickly achieved complete hematological remission and a renal response, and complete renal remission was achieved during subsequent treatment. Literature review showed that there are only a few case reports on biclonal paraprotein associated with renal amyloidosis, and only one case of renal amyloidosis associated with heavy and light-chain. In amyloidosis caused by B cell or lymphoplasmacytic clones, rituximab-based regimens are the main treatment, but hematological and organ responses are not ideal. The daratumumab combination regimen may be effective for patients with pathogenic clones of CD38⁺ B cells. Conclusions There is no consensus or guideline for the diagnosis and treatment of light-chain amyloidosis with biclonal paraprotein. Accurately identifying the pathogenic clone, determining the treatment target, and formulating individualized combination drug regimens are helpful for patients to achieve more profound remission of hematology and organs.