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Clinical characteristics and prognosis of a Chinese cohort with systemic light chain amyloidosis: a single-center study

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Abstract

Light chain amyloidosis is a plasma cell dyscrasia characterized by deposition of misfolded amyloid fibrils in tissues, leading to multi-organ dysfunction. We retrospectively analyzed 335 patients (median age, 60 years) with systemic light chain amyloidosis in the First Hospital of Peking University from 2011 to 2021. Involved organs were the kidney (92.8%), heart (57.9%), liver (12.8%) and peripheral nervous system (6.3%). Chemotherapy was administered to 55.8% (187/335) of patients, among whom 94.7% received novel agent-based regimens. Hematologic response (≥ very good partial response) was achieved in 63.4% of patients who received chemotherapy. Only 18.2% of patients received autologous hematopoietic stem cell transplant (ASCT). Among transplant-eligible patients, the overall survival of ASCT recipients was better than those who received chemotherapy only. The median overall survival of the patients with light chain amyloidosis was 77.5 months. Estimated glomerular filtration rate and Mayo 2012 stage were independent prognostic factors for overall survival in multivariate analysis. Although the younger age and high ratio of renal involvement might contribute to the favorable prognosis of this cohort, the role of novel agents and ASCT is also discernible. This study will provide a comprehensive perspective on progress in treatment of light chain amyloidosis in China.

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Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Funding

The authors declare that no funds, grants, or other support were received during the preparation of this manuscript.

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  1. Weiwei Xie and Qian Wang authors contributed to this manuscript equally.

Authors and Affiliations

  1. Department of Hematology, Peking University First Hospital, No. 7 Xi Shi Ku Street, Xi Cheng District, Beijing, 100034, People’s Republic of China

    Weiwei Xie, Qian Wang, Yuhua Sun, Xinan Cen, Hanyun Ren, Zhixiang Qiu & Yujun Dong

  2. Department of Nephrology, Peking University First Hospital, Beijing, People’s Republic of China

    Fude Zhou

  3. Laboratory of Electron Microscopy, Pathological Center, Peking University First Hospital, Beijing, People’s Republic of China

    Suxia Wang

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  1. Weiwei Xie
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Correspondence to Zhixiang Qiu or Yujun Dong.

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This study was approved by the institutional review board of PKUFH (NO. 2022yan196-002). Written informed consent was obtained from each patient in accordance with the Declaration of Helsinki.

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Xie, W., Wang, Q., Zhou, F. et al. Clinical characteristics and prognosis of a Chinese cohort with systemic light chain amyloidosis: a single-center study. Int J Hematol 118, 231–241 (2023). https://doi.org/10.1007/s12185-023-03617-8

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  • DOI: https://doi.org/10.1007/s12185-023-03617-8

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