Significant role of magnetic resonance imaging for the diagnosis and evaluation of cardiac amyloidosis in primary light chain amyloidosis

• Published in: Journal of Clinical and Experimental Hematopathology Vol. 60; no. 3; pp. 97 - 102
• Main Authors: Sekiguchi, Yasunobu, Wakabayashi, Mutsumi, Iizuka, Hiroko, Takizawa, Haruko, Sugimoto, Keiji, Sakajiri, Sakura, Inano, Tadaaki, Fukuda, Yasutaka, Hamano, Yasuharu, Tomita, Shigeki, Izumi, Hiroshi, Isogai, Hiroyuki, Okubo, Mitsuo, Nakamura, Noriko, Sawada, Tomohiro, Matsumoto, Kimihiro, Noguchi, Masaaki
• Format: Journal Article
• Language: English; Japanese
• Published: Japan The Japanese Society for Lymphoreticular Tissue Research 01.01.2020; JSLRT
• Subjects: Amyloidosis - diagnostic imaging; Amyloidosis - therapy; autologous peripheral blood stem cell transplant; cardiac amyloidosis; cardiac biomarker; cardiac MRI; Case Report; Heart - diagnostic imaging; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myocardium - pathology; primary AL amyloidosis; Stem Cell Transplantation; cardiac amyloidosis; primary AL amyloidosis; cardiac MRI; cardiac biomarker; autologous peripheral blood stem cell transplant
• ISSN: 1346-4280; 1880-9952
• DOI: 10.3960/jslrt.19040

A 47-year-old male with macroglossia presented with dyspnea on effort and chest pain at rest. Cardiac MRI revealed diffuse global subendocardial late gadolinium enhancement below the left ventricular endocardium and a dark blood pool of intracardiac contrast medium. Tongue biopsy revealed amyloid deposition, which was limited in the myocardium. He was diagnosed with primary light chain amyloidosis. His condition was stage I according to the Mayo Clinic staging system. He underwent autologous peripheral blood stem cell transplantation. On Day 10, he developed chest pain and died suddenly on Day 11. Postmortem examination revealed amyloid deposition throughout the heart.