Leung Nelson, Dispenzieri Angela, Lacy Martha Q, Kumar Shaji K, Hayman Suzanne R, Fervenza Fernando C, Cha Stephen S, Gertz Morie A
Division of Nephrology and Hypertension, Mayo Clinic Rochester, Rochester, Minnesota, USA.
Clin J Am Soc Nephrol. 2007 May;2(3):440-4. doi: 10.2215/CJN.02450706. Epub 2007 Apr 4.
Ig light chain-associated amyloidosis is a fatal plasma cell proliferative disorder that is characterized by fibril deposition in various organs. High-dose melphalan followed by autologous stem cell transplantation has been shown to improve organ dysfunction and survival. This study was undertaken to investigate factors that influence renal response. Patients who had AL amyloidosis with > or =1 g/d proteinuria and a minimum follow-up of 12 mo were recruited. Renal response was defined by >50% reduction in proteinuria with <25% decline in renal function. Hematologic response was defined as a 50% reduction in serum monoclonal protein or free light chains. Baseline characteristics were examined for relationship to renal response. Thirteen of the 135 patients were excluded for various reasons. Median follow-up was 45.4 mo. Hematologic and renal response was noted in 73 and 43.4% of the patients, respectively. Median response time for the kidney was 10 mo (1 to 40 mo). In univariate analysis, low cardiac troponin T (cTnT), higher albumin, lower proteinuria, and hematologic response were associated with renal response. In multivariate analysis, cTnT and proteinuria were predictive of renal response. Renal response was associated with a longer survival than hematologic response alone. This study showed that severe proteinuria and high cTnT negatively affected renal response after autologous stem cell transplantation. Achievement of renal response was associated with improved survival. These results suggest that early intervention with aggressive therapy is not only justified but recommended to achieve optimal response.
免疫球蛋白轻链相关淀粉样变性是一种致命的浆细胞增殖性疾病，其特征是在各个器官中出现纤维蛋白沉积。大剂量美法仑随后进行自体干细胞移植已被证明可改善器官功能障碍并提高生存率。本研究旨在调查影响肾脏反应的因素。招募了患有AL淀粉样变性且蛋白尿≥1g/d且至少随访12个月的患者。肾脏反应定义为蛋白尿减少>50%且肾功能下降<25%。血液学反应定义为血清单克隆蛋白或游离轻链减少50%。检查基线特征与肾脏反应的关系。135例患者中有13例因各种原因被排除。中位随访时间为45.4个月。分别有73%和43.4%的患者出现血液学和肾脏反应。肾脏的中位反应时间为10个月（1至40个月）。在单变量分析中，低心肌肌钙蛋白T（cTnT）、较高的白蛋白、较低的蛋白尿和血液学反应与肾脏反应相关。在多变量分析中，cTnT和蛋白尿可预测肾脏反应。与单纯血液学反应相比，肾脏反应与更长的生存期相关。本研究表明，严重蛋白尿和高cTnT对自体干细胞移植后的肾脏反应有负面影响。实现肾脏反应与生存率提高相关。这些结果表明，积极治疗的早期干预不仅合理，而且建议采用以实现最佳反应。
