Oncotarget

Research Papers:

Clinical characteristics and diagnosis of a rare case of systemic AL amyloidosis: a descriptive study

Pengguo Chen, Zhaohan Wang, Hui Liu, Donglin Liu, Zhibin Gong, Jin Qi and Jianfang Hu _

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Oncotarget. 2018; 9:24283-24290. https://doi.org/10.18632/oncotarget.25055

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Abstract

Pengguo Chen1, Zhaohan Wang1, Hui Liu1, Donglin Liu1, Zhibin Gong1, Jin Qi2 and Jianfang Hu1

1Department of Gastroenterology, Jiangxi Provincial People’s Hospital, Nanchang, Jiangxi, China

2Shanghai Institute Traumatology and Orthopaedics, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

Correspondence to:

Jianfang Hu, email: hjf5513@126.com

Keywords: amyloidosis; hepatomegaly

Received: September 19, 2017     Accepted: March 13, 2018     Published: May 11, 2018

ABSTRACT

Systemic amyloidosis is a rare disease involving multiple organs. It is difficult to establish diagnosis as the symptoms is diverse and non-specific. And without specific therapy the prognosis is very poor. We analyzed detailed clinical and laboratorial data of a 53-year-old male patient. The characteristic features included refractory pleural effusion, extraordinary hepatomegaly and cardiac failure. The illness lasted 9 months and therapy period spanned 4 months. Fine needle biopsy of liver, lung, heart, pancreas and kidney was performed. Immunohistochemistry, immunofluorescence, Congo staining and hematoxylin and eosin staining were performed. All specimens were stained pink with haematoxylin and eosin staining. Amorphous deposits of eosinophilic material were visible within the Congo red dye stained liver tissue whereas under cross-polarized light pathognomonic apple-green birefringence of amyloid deposits was visible. At last systemic AL amyloidosis diagnosis was confirmed. The report showed an unusual AL amyloidosis case in detail which would be helpful for physician in clinical work.


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