关于局部胃肠道淀粉样变性的文献系统综述:表现、管理和结果。

IF 2.3 3区 医学 Q2 HEMATOLOGY
Mariuxi Alexandra Viteri Malone, David Alfonso Alejos Castillo, Heitor Tavares Santos, Anahat Kaur, Tarek Elrafei, Lewis Steinberg, Abhishek Kumar
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引用次数: 0

摘要

目的:局部胃肠道淀粉样变性并不常见,人们对这种疾病知之甚少。目前尚无治疗局部性淀粉样变性的标准。本研究旨在评估这些患者的特征、现有治疗方法、疗效和监测情况:我们对 1962 年至 2021 年文献中报道的病例进行了系统回顾。分析对象包括英文文献中报道的胃肠道淀粉样变性患者。我们描述并总结了患者的特征、治疗方法、临床表现、结果和监测情况:结果:对已报道的临床病例进行了系统回顾,共纳入 62 例患者。在这些患者中,最常见的淀粉样蛋白沉积部位是胃(42%)。确诊年龄的中位数为64.4岁;男性(63%)与女性(37%)的发病率为2:1;腹痛是最常见的发病类型(41%),但患者也可能没有症状。单纯切除术的治愈率很高(100%)。在接受系统治疗的患者中,80%获得了完全缓解。少数病例在治疗后进行了监测,其中62%的患者仅进行了系列临床评估:据我们所知,这是首次对胃肠道淀粉样变性的文献进行系统回顾,也是规模最大的一次。淀粉样变性在男性中更为常见,单纯手术治愈率极高(100%)。对于无法切除的淀粉样变性患者,可以选择全身治疗。对这些患者进行连续的临床评估应成为标准监测护理的一部分。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A systematic review of the literature on localized gastrointestinal tract amyloidosis: Presentation, management and outcomes

Purpose

Localized gastrointestinal tract amyloidosis is uncommon and little is known regarding this entity. There is no current standard of care for the management of localized amyloidosis. The objective of this study was to evaluate the characteristics, available treatments, outcomes and surveillance of these patients.

Methods

We conducted a systematic review of cases reported in the literature from 1962 to 2021. Patients with gastrointestinal amyloidosis reported in English literature were included in the analysis. We described and summarized the patient's characteristics, treatments, clinical presentations, outcomes and surveillance.

Results

The systematic review of reported clinical cases included 62 patients. In these patients, the most common site of amyloid deposition was the stomach (42%). The median age of diagnosis is 64.4 years old; there is a 2:1 prevalence among males (63%) to females (37%); abdominal pain is the most common type of presentation (41%), although patients could also be asymptomatic. There is a high curative rate (100%) with resection alone. Among patients treated with a type of systemic therapy, 80% achieved a complete response. The minority of cases reported a type of surveillance post treatment, and among those 62% pursued serial clinical evaluations alone.

Conclusion

To our knowledge, this is the first and largest systematic review of the literature in gastrointestinal tract amyloidosis. This is more common among males and seems to have an excellent curative rate (100%) with surgery alone. Systemic therapy is an option for those with non-resectable amyloidomas. Serial clinical evaluations should be part of the standard surveillance care in these patients.

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来源期刊
CiteScore
5.50
自引率
0.00%
发文量
168
审稿时长
4-8 weeks
期刊介绍: European Journal of Haematology is an international journal for communication of basic and clinical research in haematology. The journal welcomes manuscripts on molecular, cellular and clinical research on diseases of the blood, vascular and lymphatic tissue, and on basic molecular and cellular research related to normal development and function of the blood, vascular and lymphatic tissue. The journal also welcomes reviews on clinical haematology and basic research, case reports, and clinical pictures.
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