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. 2018 Jan 15;57(2):181-187.
doi: 10.2169/internalmedicine.9206-17. Epub 2017 Nov 1.

Nationwide Survey of 741 Patients with Systemic Amyloid Light-chain Amyloidosis in Japan

Chihiro Shimazaki  1 , Hiroyuki Hata  2 , Sinsuke Iida  3 , Mitsuharu Ueda  4 , Nagaaki Katoh  5 , Yoshiki Sekijima  5 , Shuichi Ikeda  5 , Masahide Yazaki  6 , Wakaba Fukushima  7 , Yukio Ando  4
Affiliations

Nationwide Survey of 741 Patients with Systemic Amyloid Light-chain Amyloidosis in Japan

Chihiro Shimazaki et al. Intern Med. .

Abstract

Objective To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition. Methods We conducted a survey of Japanese AL amyloidosis patients, who were treated between January 1, 2012, and December 31, 2014. Results A total of 741 AL amyloidosis patients were included in this study (436 men and 305 women; median age: 65 years old, range: 31-93). The most frequently affected organ was the kidneys (n=542), followed by the heart (n=252), gastrointestinal (GI) tract (n=164), autonomic nervous system (n=131), liver (n=71), and peripheral nervous system (n=71). Diagnostic findings were most commonly detected in the GI tract (upper GI tract: 350 cases, lower GI tract: 167 cases), followed by the bone marrow and kidneys. An abdominal fat-pad biopsy was only conducted in 128 patients. Autologous stem cell transplants (ASCTs) and bortezomib were used to treat 126 and 276 patients, respectively. Conclusion The clinical features of Japanese patients with systemic AL amyloidosis are similar to those reported previously for cases in the US and Europe. Regarding treatment, a significant number of ASCTs were performed in Japan as well as in Western countries. Surprisingly, a marked number of patients received bortezomib as a treatment for AL amyloidosis.

Keywords: Japanese patients; clinical manifestation; systemic AL amyloidosis; treatment modality.

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Figures

Figure 1.
Figure 1.
Age and sex distribution at the diagnosis in 711 Japanese patients with AL amyloidosis. Thirty patients were excluded because no data were reported for them. Their median age was 65 years old, and 64% of the patients were ≥65 years old.
Figure 2.
Figure 2.
Clinical manifestations at the diagnosis in 741 Japanese patients with AL amyloidosis. Proteinuria was the most common manifestation, followed by renal dysfunction, congestive heart failure, orthostatic hypotension, diarrhea, arrhythmia, and constipation.
Figure 3.
Figure 3.
The type of detected serum M protein. λ type is more common than κ type in all immunoglobulin subclasses.
See this image and copyright information in PMC

References

    1. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med 337: 898-909, 1977. - PubMed
    1. Sipe JD, Benson MD, Buxbaum JN, et al. . Nomenclature 2014: amyloid fibril proteins and clinical classification of the amyloidosis. Amyloid 21: 221-224, 2014. - PubMed
    1. Merlini G, Stone MJ. Dangerous small B-cell clones. Blood 108: 2520-2530, 2006. - PubMed
    1. Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol 32: 45-59, 1995. - PubMed
    1. Obici L, Perfetti V, Palladini G, Moratti R, Merlini G. Clinical aspects of systemic amyloid diseases. Biochim Biophys Acta 1753: 11-22, 2005. - PubMed