Ontology highlight
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.
SUBMITTER: Houthuys JF
PROVIDER: S-EPMC9720527 | biostudies-literature | 2022 Dec
REPOSITORIES: biostudies-literature
Houthuys Jona F JF Wilmer Alexander P AP Peetermans Marijke M Meersseman Philippe P Devos Timothy T
Heliyon 20221128 12
<h4>Objective</h4>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.<h4>Results</h4>Our case presented with acute respiratory failure and a shock-like syndrome, with the need for mechanic ...[more]