Escobar Gil Tomas, Garcia Angelica Ruby, Nguyen Kimberly, Alqawasmi Malik, Borja Montes Oscar Felipe, Noronha Leonard, Babu Daniel
Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
Proc (Bayl Univ Med Cent). 2024 Jul 29;38(1):94-97. doi: 10.1080/08998280.2024.2384042. eCollection 2025.
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.
本病例报告介绍了一名25岁成年斯蒂尔病继发难治性噬血细胞性淋巴组织细胞增生症（HLH）女性患者的治疗情况。尽管最初使用了皮质类固醇和生物疗法，包括阿那白滞素和托珠单抗，但她的病情仍持续恶化，需要入住重症监护病房。经过多学科会诊，开始使用芦可替尼治疗，这是一种针对过度炎症的Janus激酶（JAK）抑制剂。值得注意的是，患者临床症状显著改善，发热消退，炎症标志物恢复正常，器官功能也得以恢复。该病例强调了芦可替尼在HLH或巨噬细胞活化综合征难治性病例中作为一种治疗选择的潜力，凸显了在难治性和具有挑战性的临床情况下探索新疗法的重要性。
