AL amyloidosis;
bone marrow plasma cells;
prognostic impact;
multiple myeloma;
PRIMARY SYSTEMIC AMYLOIDOSIS;
BRAIN NATRIURETIC PEPTIDE;
HIGH-DOSE MELPHALAN;
AL AMYLOIDOSIS;
MULTIPLE-MYELOMA;
CLINICAL-FEATURES;
PROGNOSTIC VALUE;
STAGING SYSTEM;
CARDIAC BIOMARKERS;
SURVIVAL;
D O I:
10.1080/13506129.2018.1443439
中图分类号:
Q5 [生物化学];
Q7 [分子生物学];
学科分类号:
071010 ;
081704 ;
摘要:
Objectives: Prognosis of immunoglobulin light-chain (AL) amyloidosis depends mainly on the presence of cardiac involvement and the disease burden. A higher bone marrow plasma cell (BMPC) burden has been recognized as an adverse prognostic factor. The aim of our study was to analyze the correlation between the BMPC infiltration, clinical features and outcomes in patients with AL amyloidosis.Methods: The clinical records of 79 patients with AL amyloidosis treated at a single institution.Results: Median BMPC infiltration at diagnosis was 11% and significantly correlated with the serum free light-chain difference (p<.001). Patients with more than 10% BMPCs had more frequent cardiac involvement (86 vs. 63%; p=.015), a trend towards a higher early mortality (27 vs. 11%; p=.08) and a significantly shorter progression-free survival (PFS) (median of 18 vs. 48months, p=.02) and overall survival (median of 33months vs. not reached; p=.046). In the multivariate analysis, a BMPC infiltration over 10% retained its adverse prognostic value for PFS (HR=2.26; 95% CI, 1.048-4.866; p=.038). The use of new drugs seemed to overcome the negative prognostic impact of a higher BMPC infiltration.Conclusion: Higher BMPC infiltration in AL amyloidosis might be associated with increased systemic organ damage, particularly cardiac involvement and is rarely related to the development of myeloma features.
机构:Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Hematol, 1 Shuaifuyuan, Beijing 100730, Peoples R China
Wu, Xia
Feng, Jun
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机构:Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Hematol, 1 Shuaifuyuan, Beijing 100730, Peoples R China
Feng, Jun
Cao, Xinxin
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机构:Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Hematol, 1 Shuaifuyuan, Beijing 100730, Peoples R China
Cao, Xinxin
Zhang, Lu
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机构:Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Hematol, 1 Shuaifuyuan, Beijing 100730, Peoples R China
Zhang, Lu
Zhou, Daobin
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机构:Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Hematol, 1 Shuaifuyuan, Beijing 100730, Peoples R China
Zhou, Daobin
Li, Jian
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机构:
Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Hematol, 1 Shuaifuyuan, Beijing 100730, Peoples R China
Chinese Acad Med Sci, 1 Shuaifuyuan, Beijing 100730, Peoples R ChinaPeking Union Med Coll, Peking Union Med Coll Hosp, Dept Hematol, 1 Shuaifuyuan, Beijing 100730, Peoples R China
机构:
Univ Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA
Weiss, Brendan M.
Wong, Sandy W.
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机构:
Tufts Med Ctr, John C Davis Myeloma & Amyloid Program, Boston, MA USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA
Wong, Sandy W.
Comenzo, Raymond L.
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机构:
Tufts Med Ctr, John C Davis Myeloma & Amyloid Program, Boston, MA USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA