伴有骨髓淀粉样沉积物的λ型骨髓瘤患者发生肾脏疾病的风险增加。
The risk of renal disease is increased in lambda myeloma with bone marrow amyloid deposits.
作者信息
Kozlowski Piotr, Montgomery Scott, Befekadu Rahel, Hahn-Strömberg Victoria
机构信息
Department of Medicine, School of Medical Sciences, Örebro University, Örebro, Sweden.
Clinical Epidemiology and Biostatistics, School of Medical Sciences, Örebro University, Örebro, Sweden; Clinical Epidemiology Unit, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden; Department of Epidemiology and Public Health, University College London, London, UK.
出版信息
J Blood Med. 2017 Mar 6;8:29-34. doi: 10.2147/JBM.S129516. eCollection 2017.
BACKGROUND
Light chain amyloidosis (AL) is a rare deposition disease and is present in 10-15% of patients with myeloma (MM). In contrast to symptomatic AL in MM, presence of bone marrow (BM) amyloid deposits (AD) in MM is not connected to kidney damage. Renal AD but not BM-AD occur mostly in MM with lambda paraprotein (lambda MM).
METHODS
We investigated amyloid presence in BM clots taken at diagnosis in 84 patients with symptomatic MM and compared disease characteristics in MM with kappa paraprotein (kappa MM)/lambda MM with and without BM-AD.
RESULTS
Lambda MM with BM-AD was compared with kappa MM without BM-AD, kappa MM with BM-AD, and lambda MM without BM-AD: lambda MM with BM-AD patients had a significantly higher mean creatinine level (4.23 mg/dL vs 1.69, 1.14, and 1.28 mg/dL, respectively) and a higher proportion presented with severe kidney failure (6/11 [55%] vs 6/32 [19%], 1/22 [5%], and 3/19 [16%], respectively). Proteinuria was more common in lambda MM with BM-AD patients compared with kappa MM without BM-AD patients (8/11 [73%] vs 5/32 [16%], respectively).
CONCLUSION
Kidney damage was more common in lambda MM with BM-AD indicating presence of renal AD.
背景
轻链淀粉样变性（AL）是一种罕见的沉积性疾病，在骨髓瘤（MM）患者中占10% - 15%。与MM中的有症状AL不同，MM患者骨髓（BM）淀粉样沉积物（AD）的存在与肾损伤无关。肾AD而非BM - AD大多发生于伴有λ副蛋白的MM（λMM）。
方法
我们调查了84例有症状MM患者诊断时所取BM凝块中的淀粉样蛋白情况，并比较了有或无BM - AD的κ副蛋白MM（κMM）/λMM患者的疾病特征。
结果
将伴有BM - AD的λMM与无BM - AD的κMM、有BM - AD的κMM以及无BM - AD的λMM进行比较：伴有BM - AD的λMM患者的平均肌酐水平显著更高（分别为4.23mg/dL，对比1.69mg/dL、1.14mg/dL和1.28mg/dL），且出现严重肾衰竭的比例更高（分别为6/11 [55%]，对比6/32 [19%]、1/22 [5%]和3/19 [16%]）。与无BM - AD的κMM患者相比，伴有BM - AD的λMM患者蛋白尿更为常见（分别为8/11 [73%]，对比5/32 [16%]）。
结论
伴有BM - AD的λMM中肾损伤更为常见，提示存在肾AD。
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