A diagnostic dilemma: distinguishing a sulfasalazine induced DRESS hypersensitivity syndrome from a CD30 + lymphoma in a young patient.

IF 2 Q2 EMERGENCY MEDICINE
Natalija Aleksandrova, Jonas De Rop, Frederic Camu, Ives Hubloue, Katleen Devue
{"title":"A diagnostic dilemma: distinguishing a sulfasalazine induced DRESS hypersensitivity syndrome from a CD30 + lymphoma in a young patient.","authors":"Natalija Aleksandrova, Jonas De Rop, Frederic Camu, Ives Hubloue, Katleen Devue","doi":"10.1186/s12245-024-00665-7","DOIUrl":null,"url":null,"abstract":"<p><p>Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe hypersensitivity reaction characterized by cutaneous rash, lymphadenopathy, fever, eosinophilia, leukocytosis, and life-threatening organ dysfunctions. We describe the case of a 26 year old patient admitted to the Emergency Department for DRESS syndrome after sulfasalazine treatment for rheumatoid arthritis in the right knee. Whole body computer tomography showed multiple neck, chest, and abdominal lymphadenopathy with splenomegaly, massive ascites and severe hepatic cytolysis. Serology results for Epstein-Barr Virus (EBV), influenza, measles, rubella, hepatitis A and B were negative. The histologic analysis of skin, lymph node and bone marrow biopsies could not indicate a classical Hodgkin's Disease or iatrogenic immunodeficiency/EBV-associated lymphoproliferative disorder (LPD), Hodgkin type. The relatively small caliber of the CD30 + immunoreactive blastoid cells in the lymph nodes suggested reactive immunoblasts rather than Hodgkin cells. The morphologic aspects of the lymph node biopsies with predominance of T-cells were compatible with the diagnosis of a sulfasalazine-induced DRESS syndrome as the patient had a high RegiSCAR score for DRESS. [DRESS Syndrome Foundation: Diagnosis and Treatment. (2023)] The patient's complex clinical course, marked by two hospital admissions, highlights the challenges in diagnosing and managing DRESS. This case underscores the need for individualized care, close patient monitoring, and further research to better understand DRESS's underlying mechanisms and optimal therapeutic strategies.</p>","PeriodicalId":13967,"journal":{"name":"International Journal of Emergency Medicine","volume":null,"pages":null},"PeriodicalIF":2.0000,"publicationDate":"2024-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11256461/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Journal of Emergency Medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1186/s12245-024-00665-7","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"EMERGENCY MEDICINE","Score":null,"Total":0}
引用次数: 0

Abstract

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe hypersensitivity reaction characterized by cutaneous rash, lymphadenopathy, fever, eosinophilia, leukocytosis, and life-threatening organ dysfunctions. We describe the case of a 26 year old patient admitted to the Emergency Department for DRESS syndrome after sulfasalazine treatment for rheumatoid arthritis in the right knee. Whole body computer tomography showed multiple neck, chest, and abdominal lymphadenopathy with splenomegaly, massive ascites and severe hepatic cytolysis. Serology results for Epstein-Barr Virus (EBV), influenza, measles, rubella, hepatitis A and B were negative. The histologic analysis of skin, lymph node and bone marrow biopsies could not indicate a classical Hodgkin's Disease or iatrogenic immunodeficiency/EBV-associated lymphoproliferative disorder (LPD), Hodgkin type. The relatively small caliber of the CD30 + immunoreactive blastoid cells in the lymph nodes suggested reactive immunoblasts rather than Hodgkin cells. The morphologic aspects of the lymph node biopsies with predominance of T-cells were compatible with the diagnosis of a sulfasalazine-induced DRESS syndrome as the patient had a high RegiSCAR score for DRESS. [DRESS Syndrome Foundation: Diagnosis and Treatment. (2023)] The patient's complex clinical course, marked by two hospital admissions, highlights the challenges in diagnosing and managing DRESS. This case underscores the need for individualized care, close patient monitoring, and further research to better understand DRESS's underlying mechanisms and optimal therapeutic strategies.

诊断难题:将一名年轻患者的磺胺沙拉嗪诱发 DRESS 超敏综合征与 CD30 + 淋巴瘤区分开来。
伴有嗜酸性粒细胞增多和全身症状的药物反应(DRESS)是一种严重的超敏反应,以皮疹、淋巴结病、发热、嗜酸性粒细胞增多、白细胞增多和危及生命的器官功能障碍为特征。我们描述了一例 26 岁患者因右膝盖类风湿性关节炎接受柳氮磺胺吡啶治疗后,因 DRESS 综合征被送入急诊科的病例。全身计算机断层扫描显示患者颈部、胸部和腹部多发性淋巴结病变,伴有脾脏肿大、大量腹水和严重的肝细胞溶解。爱泼斯坦-巴尔病毒(EBV)、流感、麻疹、风疹、甲型和乙型肝炎血清学检测结果均为阴性。皮肤、淋巴结和骨髓活检的组织学分析表明,该病不是典型的霍奇金病,也不是先天性免疫缺陷/EBV相关淋巴增生性疾病(LPD),霍奇金型。淋巴结中直径相对较小的 CD30 + 免疫反应性泡状细胞表明是反应性免疫母细胞而非霍奇金细胞。淋巴结活检的形态学特征以 T 细胞为主,与磺胺氯吡嗪诱发的 DRESS 综合征的诊断相符,因为患者的 DRESS RegiSCAR 评分很高。[DRESS 综合征基金会:诊断与治疗。 (2023)]该患者的临床病程复杂,曾两次入院,这凸显了诊断和处理 DRESS 所面临的挑战。该病例强调了个体化护理、密切监测患者以及进一步研究的必要性,以便更好地了解 DRESS 的潜在机制和最佳治疗策略。
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
CiteScore
4.60
自引率
0.00%
发文量
63
审稿时长
13 weeks
期刊介绍: The aim of the journal is to bring to light the various clinical advancements and research developments attained over the world and thus help the specialty forge ahead. It is directed towards physicians and medical personnel undergoing training or working within the field of Emergency Medicine. Medical students who are interested in pursuing a career in Emergency Medicine will also benefit from the journal. This is particularly useful for trainees in countries where the specialty is still in its infancy. Disciplines covered will include interesting clinical cases, the latest evidence-based practice and research developments in Emergency medicine including emergency pediatrics.
×
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