淀粉样变性患者临床特征及其预后影响因素分析

张尚艺; 张启科; 魏小芳; 冯友繁; 伏媛; 陈巧琳; 张媛媛; 张少华; 柳洁; 李晨阳

doi:10.16766/j.cnki.issn.1674-4152.004074

淀粉样变性患者临床特征及其预后影响因素分析

doi: 10.16766/j.cnki.issn.1674-4152.004074

1.
甘肃中医药大学第一临床医学院，甘肃兰州 730000
2.
甘肃省人民医院血液科，甘肃兰州 730000

基金项目:

甘肃省科技计划项目 22JR5RA660

甘肃省科技计划项目 25JRRA295

详细信息

通讯作者:
张启科，E-mail: zqk05@163.com

中图分类号: R597.2
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- 文章访问数: 7
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- 被引次数: 0
出版历程
- 收稿日期: 2024-09-24
- 网络出版日期: 2025-10-25

Analysis of clinical features and prognostic factors of amyloidosis

1.
The First Clinical Medical College of Gansu University of Traditional Chinese Medicine, Lanzhou, Gansu 730000, China

摘要

摘要: 目的淀粉样变性患者的临床表现常缺乏特异性，并且伴有多脏器功能损害，导致预后较差。本研究旨在分析淀粉样变性患者的实验室及影像学特征，并探讨该类患者预后的影响因素。方法收集甘肃省人民医院2017年7月—2023年8月收治的41例淀粉样变性患者临床资料，并对其临床特点及预后进行回顾性分析和总结。结果 41例患者的中位发病年龄为64(41~75)岁；男性27例(65.85%)，女性14例(34.15%)。28例(68.29%)患者接受化疗，6例(14.63%)患者接受自体造血干细胞移植(ASCT)，13例(31.71%)患者未治疗；对接受治疗的28例患者进行疗效评估，其中5例(17.86%)患者达到完全缓解(CR)，7例(25.00%)患者达到部分缓解(PR)，1例(3.57%)患者病情稳定(SD)，1例(3.57%)患者病情进展(PD)，7例(25.00%)患者失访，7例(25.00%)患者死亡。单因素分析显示，是否有心脏受累、是否有肾脏受累以及是否移植是影响患者总生存期的因素(P＜0.05)。多因素分析显示，有心脏、肾脏受累是影响患者总生存期的独立危险因素。41例患者中位随访时间为8(1~68)个月，中位无进展生存期(PFS)为6(1~68)个月。结论淀粉样变性以系统性轻链型淀粉样变性为主，治疗以联合化疗为主，是否有心脏受累、是否有肾脏受累与患者预后相关。对于符合移植条件的患者仍然首推ASCT。
- 淀粉样变性 /
- 轻链型淀粉样变性 /
- 临床特征 /
- 预后
Abstract: Objective The clinical manifestations of patients with amyloidosis often lack specificity and are often accompanied by multi-organ impairment, resulting in poor prognosis. Therefore, the purpose of this study was to analyze the laboratory and imaging characteristics of patients with amyloidosis and to explore the factors affecting the prognosis of these patients. Methods The clinical data of 41 patients with amyloidosis admitted to Gansu Provincial People' s Hospital from July 2017 to August 2023 were collected, and their clinical characteristics and prognosis were retrospectively analyzed and summarized. Results The median age of onset of 41 patients was 64 (41-75) years. There were 27 males (65.85%) and 14 females (34.15%). Twenty-eight patients (68.29%) received chemotherapy; 6 patients (14.63%) underwent autologous hematopoietic stem cell transplantation (ASCT). Thirteen (31.71%) patients were untreated; Efficacy was evaluated in 28 patients who received treatment, 5 (17.86%) patients had CR, 7 (25.00%) patients had PR, 1 (3.57%) had SD, 1 (3.57%) patients had PD, 7 (25.00%) patients were lost to follow-up, and 7 (25.00%) patients died. Whether there was cardiac involvement, renal involvement, and whether transplantation was performed were risk factors affecting the overall survival of patients (P < 0.05), among which cardiac and renal involvement were independent risk factors affecting the overall survival of patients. Median follow-up was 8 (1-68) months, and median PFS was 6 (1-68) months. Conclusion Amyloidosis is mainly treated with light chain amyloidosis, and the treatment is mainly combined with chemotherapy, patient prognosis is associated with age, cardiac involvement, and renal involvement. ASCT is still the first choice for transplant-eligible patients.
- Amyloidosis /
- Light chain amyloidosis /
- Clinical features /
- Prognosis

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表 1 41例淀粉样变性患者的实验室指标

Table 1. Laboratory indicators of amyloidosis patients

指标	数据
白细胞[M(P₂₅, P₇₅)，×10⁹/L]	6.25(5.00, 10.48)
中性粒细胞[M(P₂₅, P₇₅)，×10⁹/L]	4.21(3.17, 6.87)
淋巴细胞[M(P₂₅, P₇₅)，×10⁹/L]	1.33(0.95, 2.09)
单核细胞[M(P₂₅, P₇₅)，×10⁹/L]	0.45(0.32, 0.72)
血红蛋白(x±s，g/L)	130.00±31.70
血小板(x±s，×10⁹/L)	211.00±82.37
白蛋白(x±s，g/L)	27.00±8.88
球蛋白[M(P₂₅, P₇₅)，g/L]	23.20(19.54, 28.36)
Scr升高[例(%)]	12(29.27)
NT-proBNP升高[例(%)]	34(82.93)
ALP升高[例(%)]	9(21.95)
血游离κ链升高[例(%)]	13(31.71)
血游离λ链升高[例(%)]	22(53.66)
尿KAPPA链升高[例(%)]	26(63.41)
尿LAMDA链升高[例(%)]	26(63.41)
M蛋白阳性[例(%)]	30(73.17)

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表 2 淀粉样变性患者总生存时间影响因素单因素和多因素分析

Table 2. Univariate and multivariate analyses of factors affecting overall survival in patients with amyloidosis

变量	例数	单因素分析		多因素分析
变量	例数	HR(95% CI)	P值	HR(95% CI)	P值
性别(男性/女性)	27/14	0.819(-8.930~6.025)	0.591
年龄(≤60岁/＞60岁)	18/23	1.536(7.197~11.424)	0.279
是否有心脏受累(是/否)	25/16	0.591(2.942~5.618)	0.018	0.636(0.436~3.692)	0.042
是否有肾脏受累(是/否)	32/9	0.628(1.176~3.762)	0.005	0.739(1.002~2.981)	0.021
血清免疫固定电泳阳性轻链类(λ/κ)	32/9	0.813(6.269~15.228)	0.454
是否移植(是/否)	6/35	1.372(4.753~8.061)	0.037	1.539(3.649~9.027)	0.276

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