Severe ARDS due to Ruxolitinib discontinuation syndrome: case presentation and literature review. Issue 12 (December 2022)

• Record Type: Journal Article
• Authors: Houthuys, Jona F.; Wilmer, Alexander P.; Peetermans, Marijke; Meersseman, Philippe; Devos, Timothy
• Is Part Of: Heliyon. Volume 8:Issue 12(2022)
• Publication Date: 2022-12
• Subjects: Ruxolitinib -- Ruxolitinib discontinuation syndrome -- Ruxolitinib withdrawal syndrome -- Jakavi -- Jakafi -- JAK-Inhibitors -- Spontaneous spleen rupture -- Rebound cytokine storm -- ARDS -- Myelofibrosis; Research -- Periodicals; Medical sciences -- Periodicals; Natural history -- Periodicals; Social sciences -- Periodicals; Earth sciences -- Periodicals; Physical sciences -- Periodicals; 507.2
• DOI: 10.1016/j.heliyon.2022.e11782
• Languages: English
• ISSNs: 2405-8440

Abstract

Abstract: Objective: Discontinuation of Ruxolitinib (RUX), a JAK1/JAK2 inhibitor, can induce symptom-relapse and even life-threatening adverse events. Due to increasing use of RUX, this so-called RUX discontinuation syndrome (RDS) is becoming more prevalent. To create better awareness for this potentially fatal syndrome, we present a case of an adult male who developed a fatal RDS. Results: Our case presented with acute respiratory failure and a shock-like syndrome, with the need for mechanical ventilation, venovenous-extracorporeal membrane oxygenation (ECMO) and vasopressors. Respiratory symptoms quickly improved after initiation of corticosteroids, but disease course was complicated with a spontaneous spleen rupture leading to hemorrhagic shock and eventually death. Conclusion: This case report is the first case of severe RDS necessitating vv-ECMO and complicated with spleen rupture. Clinicians should be aware of this potentially lethal syndrome as it can present acutely but be effectively treated with corticosteroids and/or restarting JAK-inhibitors. Abstract : Ruxolitinib; Ruxolitinib discontinuation syndrome; Ruxolitinib withdrawal syndrome; Jakavi; Jakafi; JAK-inhibitors; Spontaneous spleen rupture; Rebound cytokine storm; ARDS; Myelofibrosis.