家族性噬血细胞淋巴组织细胞病。继发性噬血细胞综合征的鉴别诊断[j]。

C Boissy, P Velin, J F Michiels, J Diebold, P Hofman
{"title":"家族性噬血细胞淋巴组织细胞病。继发性噬血细胞综合征的鉴别诊断[j]。","authors":"C Boissy,&nbsp;P Velin,&nbsp;J F Michiels,&nbsp;J Diebold,&nbsp;P Hofman","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>We report 2 cases of familial hemophagocytic lymphohistiocytosis in two children, hétérozygous twins, born from consanguine parents. This disease is characterised by disseminated lymphohistiocytic infiltrates with hemophagocytosis, that most commonly involves bone marrow, spleen, lymph nodes, liver and central nervous system. Differential diagnosis is difficult with infection-induced hemophagocytic syndromes. The only distinguishing feature in pathology is the expression of CD21, CD30 and CD35 antigens by histiocytes. Differenciation is made by an association of clinical and pathologic characteristics: a familial history, lack of infection or neoplasm, and immunohistochemical results. Diagnostic must be rapidly made, because this disease is always fatal without treatment.</p>","PeriodicalId":75531,"journal":{"name":"Archives d'anatomie et de cytologie pathologiques","volume":"45 4","pages":"208-13"},"PeriodicalIF":0.0000,"publicationDate":"1997-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Familial hemophagocytic lymphohistiocytosis. Differential diagnosis with secondary hemophagocytic syndromes].\",\"authors\":\"C Boissy,&nbsp;P Velin,&nbsp;J F Michiels,&nbsp;J Diebold,&nbsp;P Hofman\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>We report 2 cases of familial hemophagocytic lymphohistiocytosis in two children, hétérozygous twins, born from consanguine parents. This disease is characterised by disseminated lymphohistiocytic infiltrates with hemophagocytosis, that most commonly involves bone marrow, spleen, lymph nodes, liver and central nervous system. Differential diagnosis is difficult with infection-induced hemophagocytic syndromes. The only distinguishing feature in pathology is the expression of CD21, CD30 and CD35 antigens by histiocytes. Differenciation is made by an association of clinical and pathologic characteristics: a familial history, lack of infection or neoplasm, and immunohistochemical results. Diagnostic must be rapidly made, because this disease is always fatal without treatment.</p>\",\"PeriodicalId\":75531,\"journal\":{\"name\":\"Archives d'anatomie et de cytologie pathologiques\",\"volume\":\"45 4\",\"pages\":\"208-13\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1997-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Archives d'anatomie et de cytologie pathologiques\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Archives d'anatomie et de cytologie pathologiques","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

我们报告2例家族性嗜血球性淋巴组织细胞增多症的2例患儿,他们是同父异母的混血双胞胎。本病以弥散性淋巴组织细胞浸润伴噬血细胞症为特征,最常累及骨髓、脾脏、淋巴结、肝脏和中枢神经系统。感染引起的噬血细胞综合征很难鉴别诊断。病理上唯一的区别特征是组织细胞表达CD21、CD30和CD35抗原。鉴别是由临床和病理特征:家族史,缺乏感染或肿瘤,免疫组织化学结果的关联。必须迅速作出诊断,因为这种疾病不治疗总是致命的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Familial hemophagocytic lymphohistiocytosis. Differential diagnosis with secondary hemophagocytic syndromes].

We report 2 cases of familial hemophagocytic lymphohistiocytosis in two children, hétérozygous twins, born from consanguine parents. This disease is characterised by disseminated lymphohistiocytic infiltrates with hemophagocytosis, that most commonly involves bone marrow, spleen, lymph nodes, liver and central nervous system. Differential diagnosis is difficult with infection-induced hemophagocytic syndromes. The only distinguishing feature in pathology is the expression of CD21, CD30 and CD35 antigens by histiocytes. Differenciation is made by an association of clinical and pathologic characteristics: a familial history, lack of infection or neoplasm, and immunohistochemical results. Diagnostic must be rapidly made, because this disease is always fatal without treatment.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
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