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A retrospective analysis of treatment outcomes in 45 patients with cardiac light-chain amyloidosis: a single-center experience in Japan

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Abstract

The prognosis of cardiac light-chain (AL) amyloidosis is considered to be very poor. We studied the treatment efficacy and outcomes by retrospectively analyzing the clinical results of 45 patients with cardiac AL amyloidosis treated at our hospital between September 2008 and March 2016. The group of patients analyzed included 29 males and 16 females with a median age of 68 years. Their baseline median NT-proBNP, cTnT, and dFLC were 3167 pg/ml, 0.080 ng/ml, and 286.17 mg/l, respectively. Twenty-eight patients were in Cardiac Stage (CS) III and 17 patients were in Revised Prognostic Stage (RPS) IV. At the median follow-up of 10 months, the median overall survival (OS) was 16 months and 3-year OS was 35.9%. The patients in CS III showed significantly poorer survival rate than those in CS I or II (3-year OS: 12.2% vs. 65.8%, p = 0.0115) and the patients in RPS IV showed significantly poorer survival rate than those in RPS I, II, or III (3-year OS: 11.0% vs. 53.3%, p = 0.000914). Regardless of the therapeutic approaches, patients who achieved hematological CR or cardiac organ response demonstrated significantly improved prognosis. Therefore, achievement of hematological and organ responses is important in the treatment of cardiac AL amyloidosis.

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Authors and Affiliations

  1. Department of Hematology, Japan Community Health Care Organization Kyoto Kuramaguchi Medical Center, 27 Shimofusa-cho, Kita-ku, Kyoto, 603-8151, Japan

    Shin-ichi Fuchida, Daisuke Ide, Yoko Taminishi-Katsuragawa, Saori Matsui-Maegawa, Akira Okano, Mayumi Hatsuse, Satoshi Murakami & Chihiro Shimazaki

  2. Department of Cardiology, Japan Community Health Care Organization Kyoto Kuramaguchi Medical Center, Kyoto, Japan

    Takaomi Suga, Naoki Maruyama, Yumi Iwamura, Yohei Kitamura & Yoshifumi Okawa

  3. Department of Hematology, Omihachiman Community Medical Center, Omihachiman, Japan

    Akira Okano

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  1. Shin-ichi Fuchida
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  2. Daisuke Ide
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  3. Yoko Taminishi-Katsuragawa
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  4. Takaomi Suga
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  5. Saori Matsui-Maegawa
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  6. Naoki Maruyama
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  7. Yumi Iwamura
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  9. Yoshifumi Okawa
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  11. Mayumi Hatsuse
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  12. Satoshi Murakami
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Correspondence to Shin-ichi Fuchida.

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Fuchida, Si., Ide, D., Taminishi-Katsuragawa, Y. et al. A retrospective analysis of treatment outcomes in 45 patients with cardiac light-chain amyloidosis: a single-center experience in Japan. Int J Hematol 111, 803–811 (2020). https://doi.org/10.1007/s12185-020-02835-8

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  • DOI: https://doi.org/10.1007/s12185-020-02835-8

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