Clinical Profile and Treatment in Hypereosinophilic Syndrome Variants: A Pragmatic Review

医学 嗜酸性粒细胞增多综合征 内科学 嗜酸性粒细胞 骨髓 嗜酸性粒细胞增多症 伊马替尼 胃肠病学 病理 哮喘 髓系白血病
作者
Gema Requena,Judith van den Bosch,Praveen Akuthota,Anna Kovalszki,Jonathan Steinfeld,Namhee Kwon,Melissa K. Van Dyke
出处
期刊:The Journal of Allergy and Clinical Immunology: In Practice [Elsevier]
卷期号:10 (8): 2125-2134 被引量:37
标识
DOI:10.1016/j.jaip.2022.03.034
摘要

Hypereosinophilic syndrome (HES) is a group of rare hematologic disorders leading to eosinophil-driven tissue damage and dysfunction. Better understanding of HES variants may facilitate improved patient management.To describe disease characteristics, treatment, and outcomes of patients with idiopathic (I-HES), myeloproliferative (M-HES), lymphocytic (L-HES), and chronic eosinophilic leukemia, not otherwise specified (CEL-NOS) among HES case reports and aggregate data where available.Relevant articles published between January 1, 2000, and March 20, 2020, were retrieved via PubMed; those reporting secondary, associated/reactive, overlap/single-organ, or familial HES were excluded.Of 188 articles included, 171 contained data on 347 separate HES cases (152 I-HES, 121 M-HES, 62 L-HES, 12 CEL-NOS). Based on individual data, mean age at diagnosis was 43 to 48 years for patients with all HES variants. Males accounted for 90% to 91% of M-HES/CEL-NOS and 55% to 65% of I-HES/L-HES cases. Cardiac symptoms were frequently observed for all HES variants (13%-22% of patients). Respiratory symptoms (I-HES), splenomegaly (M-HES and CEL-NOS), and skin conditions (L-HES) were also frequently observed. Bone marrow, heart, lung, spleen, liver, skin, and lymph nodes were commonly involved. Most patients with I-HES, L-HES, and CEL-NOS received corticosteroids (65%-85%), whereas most with M-HES received imatinib (81%); those with CEL-NOS also received interferon alpha (42%).Collective analysis of HES case reports supports and extends current understanding of HES variants, highlighting differences in signs and symptoms, organ involvement, and treatment approaches. Improved characterization of HES variants may facilitate the development of novel treatments.
最长约 10秒,即可获得该文献文件

科研通智能强力驱动
Strongly Powered by AbleSci AI
科研通是完全免费的文献互助平台,具备全网最快的应助速度,最高的求助完成率。 对每一个文献求助,科研通都将尽心尽力,给求助人一个满意的交代。
实时播报
lijuan发布了新的文献求助10
1秒前
www完成签到,获得积分20
3秒前
子勿语完成签到 ,获得积分10
3秒前
小葛完成签到,获得积分10
3秒前
喵阿無发布了新的文献求助10
4秒前
4秒前
4秒前
ll发布了新的文献求助10
4秒前
zhai完成签到,获得积分10
4秒前
盛夏如花发布了新的文献求助10
4秒前
6秒前
279发布了新的文献求助10
7秒前
zp_phd完成签到,获得积分10
8秒前
9秒前
9秒前
丘比特应助lijuan采纳,获得10
10秒前
科研通AI5应助Kiki采纳,获得10
10秒前
233关注了科研通微信公众号
10秒前
11秒前
梦*星愿发布了新的文献求助10
11秒前
jjj完成签到,获得积分10
11秒前
超级幻然完成签到,获得积分10
11秒前
12秒前
十一完成签到,获得积分10
12秒前
Fjj完成签到,获得积分10
13秒前
靳佳小台完成签到,获得积分10
13秒前
所所应助ll采纳,获得10
13秒前
斑马完成签到 ,获得积分10
13秒前
13秒前
Echo发布了新的文献求助10
13秒前
研友_VZG7GZ应助jiayouya123采纳,获得10
14秒前
飞翔的西红柿完成签到,获得积分10
14秒前
江湖有九哥完成签到,获得积分10
15秒前
15秒前
wzn完成签到,获得积分10
15秒前
16秒前
听见完成签到,获得积分10
16秒前
小蘑菇应助倪好采纳,获得30
16秒前
1122发布了新的文献求助10
16秒前
一只鱼完成签到,获得积分10
17秒前
高分求助中
Production Logging: Theoretical and Interpretive Elements 2700
Mechanistic Modeling of Gas-Liquid Two-Phase Flow in Pipes 2500
Kelsen’s Legacy: Legal Normativity, International Law and Democracy 1000
Handbook on Inequality and Social Capital 800
Conference Record, IAS Annual Meeting 1977 610
Interest Rate Modeling. Volume 3: Products and Risk Management 600
Interest Rate Modeling. Volume 2: Term Structure Models 600
热门求助领域 (近24小时)
化学 材料科学 生物 医学 工程类 有机化学 生物化学 物理 纳米技术 计算机科学 内科学 化学工程 复合材料 基因 遗传学 物理化学 催化作用 量子力学 光电子学 冶金
热门帖子
关注 科研通微信公众号,转发送积分 3546689
求助须知:如何正确求助?哪些是违规求助? 3123769
关于积分的说明 9356697
捐赠科研通 2822394
什么是DOI,文献DOI怎么找? 1551413
邀请新用户注册赠送积分活动 723398
科研通“疑难数据库(出版商)”最低求助积分说明 713736