N-glycosylation of monoclonal light chains on routine MASS-FIX testing is a risk factor for MGUS progression.
N-glycosylation of monoclonal light chains on routine MASS-FIX testing is a risk factor for MGUS progression.
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DOI：
10.1038/s41375-020-0940-8
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发表时间：
2020-10
期刊：
影响因子：
11.4
通讯作者：
Murray D
中科院分区：
文献类型：
--
作者：
Dispenzieri A;Larson DR;Rajkumar SV;Kyle RA;Kumar SK;Kourelis T;Arendt B;Willrcih M;Dasari S;Murray D
我们的研究组先前证明，在常规MASS-FIX检测中可检出M蛋白轻链（LC）糖基化。糖基化在免疫球蛋白轻链淀粉样变性（AL）患者中增加，并且在患者的一生中很少改变。为了确定AL和其他浆细胞疾病（PCD）的进展率，我们使用了来自Olmsted单克隆丙种球蛋白病（MGUS）筛查队列的残留血清样本。通过MASS-FIX检测了414例已知MGUS的患者，发现25例（6%）具有糖基化轻链（LC）。存活患者的中位随访时间为22.2年，有和无糖基化LC患者的20年恶性PCD进展率分别为67%（95% CI 29%，84%）和13%（95% CI 9%，18%）。进展风险与马约MGUS风险评分无关。20年时进展为AL的发生率分别为21%（95% CI 0.0，38%）和3%（95% CI 0.6%，5.5%）。总之，单克隆LC糖基化是进展为AL、骨髓瘤和其他PCD的潜在风险因素，这一观察结果可能导致早期诊断并可能降低发病率和死亡率。
Our group previously demonstrated that M-protein light chain (LC) glycosylation can be detected on routine MASS-FIX testing. Glycosylation is increased in patients with immunoglobulin light chain amyloidosis (AL) and rarely changes over the course of a patient’s lifetime. To determine the rates of progression to AL and other plasma cell disorders (PCDs), we used residual serum samples from the Olmsted monoclonal gammopathy of undetermined significance (MGUS) screening cohort. Four-hundred and fourteen patients with known MGUS were tested by MASS-FIX, and 25 (6%) were found to have glycosylated light chains (LCs). With a median follow-up of surviving patients of 22.2 years, the 20-year progression rates to a malignant PCD were 67% (95% CI 29%, 84%) and 13% (95% CI 9%, 18%) for patients with and without glycosylated LCs, respectively. The risk of progression was independent of Mayo MGUS risk score. The respective rates of progression to AL at 20-years were 21% (95% CI 0.0, 38%) and 3% (95% CI 0.6%, 5.5%). In summary, monoclonal LC glycosylation is a potent risk factor for progression to AL, myeloma, and other PCDs, an observation which could lead to earlier diagnoses and potentially reduced morbidity and mortality.
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影响因子：
168.9
作者：
Dispenzieri, Angela;Katzmann, Jerry A.;Kyle, Robert A.;Larson, Dirk R.;Melton, L. Joseph, III;Colby, Colin L.;Therneau, Terry M.;Clark, Raynell;Kumar, Shaji K.;Bradwell, Arthur;Fonseca, Rafael;Jelinek, D. F.;Rajkumar, S. Vincent
通讯作者：
Rajkumar, S. Vincent
影响因子：
12.8
作者：
Milani, Paolo;Murray, David L.;Dispenzieri, Angela
通讯作者：
Dispenzieri, Angela
DOI：
10.1515/bchm3.1985.366.2.617
发表时间：
1985-01-01
期刊：
BIOLOGICAL CHEMISTRY HOPPE-SEYLER
影响因子：
--
作者：
TOFT, KG;SLETTEN, K;HUSBY, G
通讯作者：
HUSBY, G
影响因子：
9.3
作者：
Mills, John R.;Kohlhagen, Mindy C.;Murray, David L.
通讯作者：
Murray, David L.
DOI：
10.3109/13506120009146835
发表时间：
2000-09-01
期刊：
AMYLOID-INTERNATIONAL JOURNAL OF EXPERIMENTAL AND CLINICAL INVESTIGATION
影响因子：
--
作者：
Stevens, FJ
通讯作者：
Stevens, FJ