UNSTRUCTURED
Abstract
Purpose: To describe the clinical characteristics of Chinese patients with immunoglobulin light chain (AL) amyloidosis.
Experimental Design: searches were made in MEDLINE, EMBASE, and CNKI. Articles (interventional and non-interventional) that reported Chinese patients with AL Amyloidosis were included in this study. The included characteristics were age, gender, λ type or κ type, body mass index (BMI), the time from onset of symptoms to diagnosis, Mayo cardiac staging, primary or secondary, organ involvement etc.
Results: Sixty-seven articles reporting 5022 patients were included. The weighted average age of Chinese patients with AL amyloidosis was 57 years, and 61.2% of patients were males. 80.0%, 14.1% and 5.9% of patients were λ type, κ type and type unclear, respectively. The mean BMI was 22.82 ± 3.24 kg/m2. The time range from onset of symptoms to diagnosis was between 6 and 12 months. The proportion of patients at Mayo (2004) stage I, Ⅱ and Ⅲ was 24.6%, 34.3% and 41.1% (respectively). The proportion of patients at Mayo (2012) stage I, Ⅱ, Ⅲ and Ⅳ was 33.6%, 24.7%, 25.2% and 16.6% (respectively). 31.3% of patients were in New York Heart Association (NYHA) grade III-IV. 20.1% of patients were AL amyloidosis with concurrent multiple myeloma. 79.3% of patients were categorized as having more than one organ involvement. The involvement of the kidney, heart, skin/soft tissue and digestive system accounted for 84.3%, 62.5%, 41.5%, and 34.4% of patients, respectively.
Conclusions: This scoping review suggests that most patients with AL amyloidosis were at an advanced stage when diagnosed in China. Epidemiological studies with a larger sample size are needed to comprehensively investigate the AL amyloidosis patients’ characteristics in the Chinese population.