Long term outcomes in monoclonal gammopathy of renal significance

被引:21
作者
Khera, Akhil [1 ]
Panitsas, Fotios [2 ]
Djebbari, Faouzi [1 ]
Kimberger, Katja [3 ]
Stern, Simon [4 ]
Quinn, John [5 ]
Rabin, Neil [6 ]
Kothari, Jaimal [1 ,7 ]
Alchi, Bassam [3 ]
Haynes, Richard [8 ]
Winearls, Christopher [8 ]
Roberts, Ian [9 ]
Ramasamy, Karthik [1 ,7 ]
机构
[1] Oxford Univ Hosp NHS Fdn Trust UK, Dept Haematol, Oxford, England
[2] Larissa Univ Hosp, Dept Hematol, Larisa, Greece
[3] Royal Berkshire NHS Fdn Trust, Dept Nephrol, Reading, Berks, England
[4] Epsom & St Helier Univ Hosp NHS Trust, Dept Haematol, Carshalton, Surrey, England
[5] Beaumont Hosp, Dept Haematol, Dublin, Ireland
[6] Univ Coll London Hosp NHS Fdn Trust, Dept Haematol, London, England
[7] NIHR BRC Blood Theme, Oxford, England
[8] Oxford Univ Hosp NHS Fdn Trust UK, Oxford Kidney Unit, Oxford, England
[9] Oxford Univ Hosp NHS Fdn Trust, Dept Cellular Pathol, Oxford, England
关键词
monoclonal gammopathy of renal significance; light chain deposition disease; renal survival; chronic kidney disease; bortezomib; IMMUNOGLOBULIN DEPOSITION DISEASE; DIAGNOSIS; CRITERIA;
D O I
10.1111/bjh.15987
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Unlike AL amyloid and cast nephropathy, the long-term outcomes of monoclonal gammopathy of renal significance (MGRS) patients with other renal histopathologies remain unclear. It is uncertain if early intervention improves renal outcomes, because of a lack of evidence from prospective studies. In this retrospective study, we examined outcomes of 41 MGRS patients treated between 2004 and 2017 across five centres: four in the UK and one in the Republic of Ireland. The primary outcome measure was renal survival estimated by Kaplan-Meier product-limit method. Thirty-three patients (80 center dot 5%) were kappa light chain (LC) restricted. Twenty-seven patients (65 center dot 9%) presented with LC deposition disease on renal histology. At 24 months follow-up, estimated renal survival was 81 center dot 6% for the whole cohort. The estimated overall survival was 80 center dot 3% at 48 months. At 24 months, patients who had chronic kidney disease (CKD) stage 2-3b at diagnosis showed an estimated renal survival of 100% compared to 80 center dot 7% in those with CKD stage 4-5 at diagnosis (P = 0 center dot 04). Poorer outcomes in MGRS patients were historically attributed to delayed diagnosis due to small plasma cell clones, as well as the need for renal biopsy.
引用
收藏
页码:706 / 716
页数:11
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