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Clinical and echocardiographic characteristics for differentiating between transthyretin-related and light-chain cardiac amyloidoses

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Abstract

Differential diagnosis between transthyretin (TTR) and immunoglobulin light-chain (AL) cardiac amyloidoses is essential due to significantly different prognoses and therapeutic options. Therefore, clinical characteristics of patients with biopsy-proven cardiac amyloidosis were investigated to differentiate TTR from AL amyloidosis. From September 2006 to May 2014, 46 patients were confirmed to have cardiac amyloidosis (TTR, n = 28; AL, n = 18) in our institute. The median age of patients with TTR amyloidosis was 78 years (range 61–90) with 27 (96 %) males, while that of patients with AL amyloidosis was 66 (range 52–76) with 12 (67 %) males. There were no statistically significant differences in echocardiographic findings regarding left ventricular (LV) systolic function or diastolic dysfunction between the two groups. Interestingly, serum brain natriuretic peptide (BNP) levels in patients with AL amyloidosis were significantly higher than those in TTR amyloidosis patients. In contrast, the LV wall was significantly thicker in patients with TTR amyloidosis than in those with AL amyloidosis. Therefore, the ratio of BNP to LV mass index (LVMI) at presentation in AL amyloidosis patients was significantly higher than that in TTR patients (6.7 vs 2.9, p = 0.0006). A BNP-LVMI ratio of less than 3.5 had a diagnostic sensitivity and specificity for TTR amyloidosis of 71 and 83 %, respectively. One-year overall survival was 88.7 % in the patients with TTR amyloidosis and 23.7 % in the patients with AL amyloidosis. Our analysis indicates that the BNP-LVMI ratio, as well as age and sex, may be useful parameters for distinguishing TTR from AL cardiac amyloidosis.

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Acknowledgments

The authors are grateful to Dr. Yoshinobu Hoshii, Department of Pathology, Yamaguchi University School of Medicine, for performing the immunohistochemical analyses.

Conflict of interest

The authors report no potential conflicts of interest.

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Authors and Affiliations

  1. Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan

    Minako Mori, Oju Katayama, Tomoya Kitagawa, Yuya Sasaki, Takashi Onaka, Akihito Yonezawa & Kazunori Imada

  2. Department Cardiology, Kokura Memorial Hospital, Kitakyushu, Japan

    Yoshimori An & Kenji Ando

  3. Diagnostic Pathology, Kokura Memorial Hospital, Kitakyushu, Japan

    Kenichiro Murata & Tadaaki Yokota

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  1. Minako Mori
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  2. Yoshimori An
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Correspondence to Kazunori Imada.

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Mori, M., An, Y., Katayama, O. et al. Clinical and echocardiographic characteristics for differentiating between transthyretin-related and light-chain cardiac amyloidoses. Ann Hematol 94, 1885–1890 (2015). https://doi.org/10.1007/s00277-015-2466-0

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  • DOI: https://doi.org/10.1007/s00277-015-2466-0

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