Tomas Escobar Gil, Angelica Ruby Garcia, Kimberly Nguyen, Malik Alqawasmi, Oscar Felipe Borja Montes, Leonard Noronha, Daniel Babu
{"title":"打破风暴:鲁索利替尼作为难治性噬血细胞淋巴组织细胞增多症伴巨噬细胞活化综合征的生命线。","authors":"Tomas Escobar Gil, Angelica Ruby Garcia, Kimberly Nguyen, Malik Alqawasmi, Oscar Felipe Borja Montes, Leonard Noronha, Daniel Babu","doi":"10.1080/08998280.2024.2384042","DOIUrl":null,"url":null,"abstract":"<p><p>This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated. Remarkably, the patient exhibited significant clinical improvement with resolution of fever, normalization of inflammatory markers, and organ function recovery. This case underscores the potential of ruxolitinib as a therapeutic option in refractory cases of HLH or macrophage activation syndrome, highlighting the importance of exploring novel therapies in refractory and challenging clinical scenarios.</p>","PeriodicalId":8828,"journal":{"name":"Baylor University Medical Center Proceedings","volume":"38 1","pages":"94-97"},"PeriodicalIF":0.0000,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11657089/pdf/","citationCount":"0","resultStr":"{\"title\":\"Breaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.\",\"authors\":\"Tomas Escobar Gil, Angelica Ruby Garcia, Kimberly Nguyen, Malik Alqawasmi, Oscar Felipe Borja Montes, Leonard Noronha, Daniel Babu\",\"doi\":\"10.1080/08998280.2024.2384042\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated. Remarkably, the patient exhibited significant clinical improvement with resolution of fever, normalization of inflammatory markers, and organ function recovery. This case underscores the potential of ruxolitinib as a therapeutic option in refractory cases of HLH or macrophage activation syndrome, highlighting the importance of exploring novel therapies in refractory and challenging clinical scenarios.</p>\",\"PeriodicalId\":8828,\"journal\":{\"name\":\"Baylor University Medical Center Proceedings\",\"volume\":\"38 1\",\"pages\":\"94-97\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11657089/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Baylor University Medical Center Proceedings\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1080/08998280.2024.2384042\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Baylor University Medical Center Proceedings","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1080/08998280.2024.2384042","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Breaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.
This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated. Remarkably, the patient exhibited significant clinical improvement with resolution of fever, normalization of inflammatory markers, and organ function recovery. This case underscores the potential of ruxolitinib as a therapeutic option in refractory cases of HLH or macrophage activation syndrome, highlighting the importance of exploring novel therapies in refractory and challenging clinical scenarios.