Severe ARDS due to Ruxolitinib discontinuation syndrome: case presentation and literature review

Jona F Houthuys; Alexander P Wilmer; Marijke Peetermans; Philippe Meersseman; Timothy Devos

doi:10.1016/j.heliyon.2022.e11782

Severe ARDS due to Ruxolitinib discontinuation syndrome: case presentation and literature review

Heliyon. 2022 Nov 28;8(12):e11782. doi: 10.1016/j.heliyon.2022.e11782. eCollection 2022 Dec.

Authors

Jona F Houthuys^{1

2}, Alexander P Wilmer^{1

2}, Marijke Peetermans^{1

2}, Philippe Meersseman^{1

2}, Timothy Devos^{2

3}

Affiliations

¹ Medical Intensive Care, UZ Leuven, Gasthuisberg, Leuven, Vlaams Brabant, Belgium.
² Department of Health, KU Leuven University, Leuven, Vlaams Brabant, Belgium.
³ Department of Haematology, UZ Leuven, Gasthuisberg, Leuven, Vlaams Brabant, Belgium.

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.

Keywords: ARDS; JAK-Inhibitors; Jakafi; Jakavi; Myelofibrosis; Rebound cytokine storm; Ruxolitinib; Ruxolitinib discontinuation syndrome; Ruxolitinib withdrawal syndrome; Spontaneous spleen rupture.

Publication types

Case Reports