文献类型：期刊文献

英文题名：Waldenstr?m macroglobulinemia complicated with AL λ - type amyloid nephropathy: a case report and literature review

作者：Ma, Yuan[1];Li, Taotao[1];Li, Xiaoli[2]

第一作者：马毅;马艳

通信作者：Li, XL[1]

机构：[1]Gansu Univ Chinese Med, Lanzhou, Peoples R China;[2]Gansu Prov Hosp, Dept Nephrol, Lanzhou, Peoples R China

第一机构：甘肃中医药大学

通信机构：[1]corresponding author), Gansu Prov Hosp, Dept Nephrol, Lanzhou, Peoples R China.

年份：2026

卷号：13

外文期刊名：FRONTIERS IN MEDICINE

收录：;WOS:【SCI-EXPANDED(收录号:WOS:001751955300001)】；

基金：The author(s) declared that financial support was received for this work and/or its publication. This work was supported by the Natural Science Foundation of Gansu Province (22JR5RA654).

语种：英文

外文关键词：AL-type amyloidosis; IgM; MYD88-L265P mutation; nephrotic syndrome; prognostic analysis; Waldenstr & ouml;m macroglobulinemia; lambda light chain

摘要：Waldenstr & ouml;m macroglobulinemia (WM) is a rare CD20-positive B-cell non-Hodgkin lymphoma. It is characterized by lymphoplasmacytic infiltration in the bone marrow and abnormal monoclonal IgM secretion. WM complicated by renal amyloidosis is uncommon but associated with rapid progression of organ damage. Accurate identification of pathogenic factors and individualized treatments are essential to improve prognosis. This paper reports the case of a 75-year-old female who initially presented with facial edema and bilateral lower-extremity edema lasting 2 days. Additional symptoms included foamy urine, frequent urination, and urgency. Laboratory tests revealed significant proteinuria (24-h urinary protein, 6.11 g), hypoalbuminemia (serum albumin, 20.07 g/L), and impaired renal function (serum creatinine, 129.90 mu mol/L; eGFR, 45.2 mL/min/1.73 m2). Immunofixation electrophoresis detected monoclonal IgM-lambda immunoglobulin in both serum and urine. Bone marrow biopsy demonstrated clonal lymphoplasmacytic infiltration (32%), and genetic testing revealed a positive MYD88-L265P mutation (mutation frequency, 28.5%). Renal biopsy indicated diffuse deposition of lambda light chains predominantly in the glomerular mesangium. Ultrastructural examination revealed amyloid fibrils, confirming the diagnosis of AL lambda - type amyloid nephropathy. Chemotherapy with rituximab, cyclophosphamide, and dexamethasone (RCD regimen), combined with symptomatic supportive therapy, resulted in temporary improvement of clinical symptoms and laboratory parameters. However, the disease progressed rapidly. The patient died on Jan 28, 2024, approximately 3 months after discharge, due to multiple organ failure involving cardiac, renal, and respiratory dysfunction. Based on relevant literature and the Chinese Expert Consensus on Diagnosis and Treatment of Lymphoplasmacytic Lymphoma (LPL)/WM (2022 Edition) [Abstract 3], this paper discusses diagnostic criteria, differentiation of pathogenic components, treatment strategies, and prognostic factors. These findings may provide clinical guidance for similar rare cases.