Primary Immunodeficiency and Thrombocytopenia.

IF 4.3 4区 医学 Q2 IMMUNOLOGY International Reviews of Immunology Pub Date : 2022-01-01 Epub Date: 2021-01-19 DOI:10.1080/08830185.2020.1868454
Maryam Mohtashami, Azadehsadat Razavi, Hassan Abolhassani, Asghar Aghamohammadi, Reza Yazdani
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引用次数: 2

Abstract

Primary immunodeficiency (PID) or Inborn errors of immunity (IEI) refers to a heterogeneous group of disorders characterized by immune system impairment. Although patients with IEI manifest highly variable symptoms, the most common clinical manifestations are recurrent infections, autoimmunity and malignancies. Some patients present hematological abnormality including thrombocytopenia due to different pathogenic mechanisms. This review focuses on primary and secondary thrombocytopenia as a complication, which can occur in IEI. Based on the International Union of Immunological Societies phenotypic classification for IEI, the several innate and adaptive immunodeficiency disorders can lead to thrombocytopenia. This review, for the first time, describes manifestation, mechanism and therapeutic modalities for thrombocytopenia in different classes of IEI.

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原发性免疫缺陷和血小板减少症。
原发性免疫缺陷(PID)或先天性免疫缺陷(IEI)是指以免疫系统损伤为特征的异质性疾病。虽然IEI患者的症状变化很大,但最常见的临床表现是反复感染、自身免疫和恶性肿瘤。由于致病机制不同,部分患者出现包括血小板减少在内的血液学异常。这篇综述的重点是原发性和继发性血小板减少症作为并发症,可发生在IEI。根据国际免疫学会联合会对IEI的表型分类,几种先天性和适应性免疫缺陷疾病可导致血小板减少症。本文首次综述了不同类型IEI中血小板减少症的表现、机制和治疗方式。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
11.00
自引率
4.00%
发文量
24
期刊介绍: This review journal provides the most current information on basic and translational research in immunology and related fields. In addition to invited reviews, the journal accepts for publication articles and editorials on relevant topics proposed by contributors. Each issue of International Reviews of Immunology contains both solicited and unsolicited review articles, editorials, and ''In-this-Issue'' highlights. The journal also hosts reviews that position the authors'' original work relative to advances in a given field, bridging the gap between annual reviews and the original research articles. This review series is relevant to all immunologists, molecular biologists, microbiologists, translational scientists, industry researchers, and physicians who work in basic and clinical immunology, inflammatory and allergic diseases, vaccines, and additional topics relevant to medical research and drug development that connect immunology to disciplines such as oncology, cardiovascular disease, and metabolic disorders. Covered in International Reviews of Immunology: Basic and developmental immunology (innate and adaptive immunity; inflammation; and tumor and microbial immunology); Clinical research (mechanisms of disease in man pertaining to infectious diseases, autoimmunity, allergy, oncology / immunology); and Translational research (relevant to biomarkers, diagnostics, vaccines, and drug development).
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