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极高危原发性轻链型淀粉样变患者的临床特征和预后分析

OBJECTIVE: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. METHODS: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2...

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Formato: Online Artículo Texto
Lenguaje:English
Publicado: Editorial office of Chinese Journal of Hematology 2017
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7354162/
https://www.ncbi.nlm.nih.gov/pubmed/28279033
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2017.02.005
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collection PubMed
description OBJECTIVE: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. METHODS: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A ‘very high risk’ group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4. RESULTS: Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients. The median age at diagnosis was 57 (20–84) years, and 22 patients (64.7%) were male. All 34 patients were diagnosed with cardiac involvement, multi-organ involvement was observed in 15 patients (44.1%), and 27 (81.8%) had New York Heart Association Class Ⅲ or Ⅳ. Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 µg/L, 11 733 ng/L, and 403 mg/L, respectively. Eight (24.2%) had more than 10% plasma cell on the bone marrow aspirate. Sixteen (47.1%) patients received bortezomib based chemotherapy and overall hematologic response rate was 58.3%. Median overall survival (OS) was 4 months. The estimated OS at 3, 6, 12, and 24 months was 51.3%, 44.0%, 35.2%, and 29.6%, respectively. Fourteen (41.2%) patients died within 3 months after the diagnosis. The estimated 1-year survival rate for the patients who got hematologic response, without hematologic response, and palliative treatment was 90.9%, 11.1%, and 0, respectively (P<0.001). CONCLUSION: Patients with very high risk pAL had very poor prognosis and the early death rate remained high. Those patients who obtained hematologic remission would have significantly better outcomes.
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spelling pubmed-73541622020-07-16 极高危原发性轻链型淀粉样变患者的临床特征和预后分析 Zhonghua Xue Ye Xue Za Zhi 论著 OBJECTIVE: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. METHODS: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A ‘very high risk’ group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4. RESULTS: Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients. The median age at diagnosis was 57 (20–84) years, and 22 patients (64.7%) were male. All 34 patients were diagnosed with cardiac involvement, multi-organ involvement was observed in 15 patients (44.1%), and 27 (81.8%) had New York Heart Association Class Ⅲ or Ⅳ. Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 µg/L, 11 733 ng/L, and 403 mg/L, respectively. Eight (24.2%) had more than 10% plasma cell on the bone marrow aspirate. Sixteen (47.1%) patients received bortezomib based chemotherapy and overall hematologic response rate was 58.3%. Median overall survival (OS) was 4 months. The estimated OS at 3, 6, 12, and 24 months was 51.3%, 44.0%, 35.2%, and 29.6%, respectively. Fourteen (41.2%) patients died within 3 months after the diagnosis. The estimated 1-year survival rate for the patients who got hematologic response, without hematologic response, and palliative treatment was 90.9%, 11.1%, and 0, respectively (P<0.001). CONCLUSION: Patients with very high risk pAL had very poor prognosis and the early death rate remained high. Those patients who obtained hematologic remission would have significantly better outcomes. Editorial office of Chinese Journal of Hematology 2017-02 /pmc/articles/PMC7354162/ /pubmed/28279033 http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2017.02.005 Text en 2017年版权归中华医学会所有 http://creativecommons.org/licenses/by-nc-sa/3.0/ This work is licensed under a Creative Commons Attribution 3.0 License (CC-BY-NC). The Copyright own by Publisher. Without authorization, shall not reprint, except this publication article, shall not use this publication format design. Unless otherwise stated, all articles published in this journal do not represent the views of the Chinese Medical Association or the editorial board of this journal.
spellingShingle 论著
极高危原发性轻链型淀粉样变患者的临床特征和预后分析
title 极高危原发性轻链型淀粉样变患者的临床特征和预后分析
title_full 极高危原发性轻链型淀粉样变患者的临床特征和预后分析
title_fullStr 极高危原发性轻链型淀粉样变患者的临床特征和预后分析
title_full_unstemmed 极高危原发性轻链型淀粉样变患者的临床特征和预后分析
title_short 极高危原发性轻链型淀粉样变患者的临床特征和预后分析
title_sort 极高危原发性轻链型淀粉样变患者的临床特征和预后分析
topic 论著
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7354162/
https://www.ncbi.nlm.nih.gov/pubmed/28279033
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2017.02.005
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