关键词: 轻链型淀粉样变性, 全切片扫描分析, 临床特点, 预后

Abstract:

Ｏｂｊｅｃｔｉｖｅ：To explore the location and severity of amyloid deposition on kidney and compare them with clinical features in patients with lightchain amyloidosis (AL).
Ｍｅｔｈｏｄｏｌｏｇｙ：The clinicopathological data of 259 patients with kidney biopsyproven AL amyloidsis were retrospectively analyzed. The presence of amyloid was identified by positive congo red staining from digital whole slide imaging. Areas with amyloid deposition and glomerular, vascular and total tissue area were measured by digital WSI. Areas of amyloid deposition per total glomerular, vascular, interstitial and total tissue were calculated respectively using Aperio software.
Ｒｅｓｕｌｔｓ：They were 147 males and 102 females, with a median age of 57 years old. The patients frequently presented with edema and fatigue. The most common involved organ was kidney (100%), followed by gastrointestinal tract (711%) and heart (432%). The median disease duration was 7 months. The mean level of proteinuria was 480±315 g/24h. 50 patients had renal insufficiency. The median time of followup was 223 months, and the mean patient survival time was 242 months. The 1, 2, 3 and 5year cumulative patient survival rates were 818%,641%,524% and 296%, and cumulative renal survival rates were 908%,823%,768% and 478%. The numbers of patients who had amyloid deposition in glomerular, vascular and interstitial areas were 258 (996%), 239 (927%) and 165 (502%) respectively. The rates of glomerular, vascular, interstitial and total tissue areas amyloid deposits were 118±114%, 141±141%, 334±536% and 425±577% respectively, which were associated with several clinical parameters. The rate of glomerular amyloid deposits, age, urinary NacetylBDglucosaminidase to creatinine ratios (NAG) and retinolbinding protein (RBP) were important risk factors of patient survival. The rates of vascular, interstitial, total tissue areas amyloid deposits, serum creatinine (SCr) and RBP were important risk factors of renal survival.
Ｃｏｎｃｌｕｓｉｏｎ：The digital whole slide imaging is convenient, practical and feasible in evaluation of amyloid deposits. The rates of glomerular and vascular amyloid deposit were same and higher than that in interstitial areas. Proteinuria and SCr were associated with amyloid deposition. The amyloid deposition was related with patient survival and renal survival.

Key words: lightchain amyloidosis, whole scanning image, clinical manifestation, prognosis