Hairy cell leukemia - etiopathogenesis, diagnosis and modern therapeutic approach.

Katarzyna Maćkowiak, Magdalena Jankowiak, Karolina Szewczyk-Golec, Iga Hołyńska-Iwan
{"title":"Hairy cell leukemia - etiopathogenesis, diagnosis and modern therapeutic approach.","authors":"Katarzyna Maćkowiak, Magdalena Jankowiak, Karolina Szewczyk-Golec, Iga Hołyńska-Iwan","doi":"10.11613/BM.2024.020502","DOIUrl":null,"url":null,"abstract":"<p><p>Hairy cell leukemia (HCL) represents 2% of all leukemia cases, with men aged above 55 years being the most affected. The most common symptoms of this type of leukemia include splenomegaly, monocytopenia, and neutropenia. In the basic blood count examination, leukopenia with monocytopenia and granulocytopenia, as well as aplastic anemia and/or thrombocytopenia occur. The mutation of β-rapidly accelerated fibrosarcoma (<i>BRAF</i>) proto-oncogene, which can be found in nearly 100% of patients, is an important feature of HCL. Immunophenotypic analysis of the HCL cells reveals high expression of B-lineage antigens, including CD19, CD20, and CD22. Additionally, CD11c, CD25, CD103, and CD123 belong to specific markers of HCL. Lactate dehydrogenase activity and β-2-microglobulin concentration are also important in the patient's assessment. The differential diagnosis between HCL, hairy cell leukemia variant (HCL-V) and splenic marginal zone lymphoma (SMZL) is of first importance. Currently, the main treatment for HCL involves the use of purine analogues, excluding pregnant women, individuals with severe infections, and those with relapsing HCL.</p>","PeriodicalId":94370,"journal":{"name":"Biochemia medica","volume":"34 2","pages":"020502"},"PeriodicalIF":0.0000,"publicationDate":"2024-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11177658/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Biochemia medica","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.11613/BM.2024.020502","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

Abstract

Hairy cell leukemia (HCL) represents 2% of all leukemia cases, with men aged above 55 years being the most affected. The most common symptoms of this type of leukemia include splenomegaly, monocytopenia, and neutropenia. In the basic blood count examination, leukopenia with monocytopenia and granulocytopenia, as well as aplastic anemia and/or thrombocytopenia occur. The mutation of β-rapidly accelerated fibrosarcoma (BRAF) proto-oncogene, which can be found in nearly 100% of patients, is an important feature of HCL. Immunophenotypic analysis of the HCL cells reveals high expression of B-lineage antigens, including CD19, CD20, and CD22. Additionally, CD11c, CD25, CD103, and CD123 belong to specific markers of HCL. Lactate dehydrogenase activity and β-2-microglobulin concentration are also important in the patient's assessment. The differential diagnosis between HCL, hairy cell leukemia variant (HCL-V) and splenic marginal zone lymphoma (SMZL) is of first importance. Currently, the main treatment for HCL involves the use of purine analogues, excluding pregnant women, individuals with severe infections, and those with relapsing HCL.

毛细胞白血病--发病机制、诊断和现代治疗方法。
毛细胞白血病(HCL)占所有白血病病例的 2%,55 岁以上的男性患者最多。这类白血病最常见的症状包括脾肿大、单核细胞减少和中性粒细胞减少。在基本血细胞计数检查中,会出现白细胞减少伴单核细胞减少和粒细胞减少,以及再生障碍性贫血和/或血小板减少。β-快速加速纤维肉瘤(BRAF)原癌基因的突变是 HCL 的一个重要特征,几乎 100%的患者都存在这种突变。HCL 细胞的免疫表型分析显示,包括 CD19、CD20 和 CD22 在内的 B 系抗原高表达。此外,CD11c、CD25、CD103 和 CD123 也属于 HCL 的特异性标记。乳酸脱氢酶活性和β-2-微球蛋白浓度对患者的评估也很重要。HCL、毛细胞白血病变异型(HCL-V)和脾边缘区淋巴瘤(SMZL)之间的鉴别诊断至关重要。目前,HCL 的主要治疗方法是使用嘌呤类似物,但孕妇、严重感染者和复发性 HCL 患者除外。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信