Vos Josephine M, Gustine Joshua, Rennke Helmut G, Hunter Zachary, Manning Robert J, Dubeau Toni E, Meid Kirsten, Minnema Monique C, Kersten Marie-Jose, Treon Steven P, Castillo Jorge J
Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA.
Department of Internal Medicine/Haematology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Br J Haematol. 2016 Nov;175(4):623-630. doi: 10.1111/bjh.14279. Epub 2016 Jul 29.
The incidence and prognostic impact of nephropathy related to Waldenström macroglobulinaemia (WM) is currently unknown. We performed a retrospective study to assess biopsy-confirmed WM-related nephropathy in a cohort of 1391 WM patients seen at a single academic institution. A total of 44 cases were identified, the estimated cumulative incidence was 5·1% at 15 years. There was a wide variation in kidney pathology, some directly related to the WM: amyloidosis (n = 11, 25%), monoclonal-IgM deposition disease/cryoglobulinaemia (n = 10, 23%), lymphoplasmacytic lymphoma infiltration (n = 8, 18%), light-chain deposition disease (n = 4, 9%) and light-chain cast nephropathy (n = 4, 9%), and some probably related to the WM: thrombotic microangiopathy (TMA) (n = 3, 7%), minimal change disease (n = 2, 5%), membranous nephropathy (n = 1, 2%) and crystal-storing tubulopathy (n = 1, 2%). The median overall survival in patients with biopsy-confirmed WM-related nephropathy was 11·5 years, shorter than for the rest of the cohort (16 years, P = 0·03). Survival was better in patients with stable or improved renal function after treatment (P = 0·05). Based on these findings, monitoring for renal disease in WM patients should be considered and a kidney biopsy pursued in those presenting with otherwise unexplained renal failure and/or nephrotic syndrome.
与华氏巨球蛋白血症（WM）相关的肾病的发病率及其对预后的影响目前尚不清楚。我们进行了一项回顾性研究，以评估在一家学术机构就诊的1391例WM患者队列中经活检确诊的WM相关肾病。共识别出44例病例，15年时的估计累积发病率为5.1%。肾脏病理存在广泛差异，有些与WM直接相关：淀粉样变性（n = 11，25%）、单克隆IgM沉积病/冷球蛋白血症（n = 10，23%）、淋巴浆细胞淋巴瘤浸润（n = 8，18%）、轻链沉积病（n = 4，9%）和轻链管型肾病（n = 4，9%），有些可能与WM相关：血栓性微血管病（TMA）（n = 3，7%）、微小病变病（n = 2，5%）、膜性肾病（n = 1，2%）和晶体贮积性肾小管病（n = 1，2%）。经活检确诊的WM相关肾病患者的中位总生存期为11.5年，短于队列中的其他患者（16年，P = 0.03）。治疗后肾功能稳定或改善的患者生存期更好（P = 0.05）。基于这些发现，应考虑对WM患者进行肾脏疾病监测，对于出现不明原因肾衰竭和/或肾病综合征的患者应进行肾活检。
