Ruxolitinib as adjunctive therapy for secondary hemophagocytic lymphohistiocytosis: A case series.
Eur J Haematol
; 106(5): 654-661, 2021 May.
Article
in English
| MEDLINE | ID: covidwho-1057986
ABSTRACT
INTRODUCTION:
Hemophagocytic lymphohistiocytosis (HLH) is a cytokine storm syndrome associated with mortality rates of up to 88%. Standard therapy with high-dose glucocorticoids and etoposide used in adults is extrapolated from pediatric trials, with significant toxicity in older patients and those with poor performance status. The JAK1/2 inhibitor ruxolitinib has recently gained attention as a treatment option for HLH due to its broad cytokine-modulating abilities and safety profile. Herein we report our center's experience using ruxolitinib in the treatment of adult-onset secondary HLH. CASE SERIES We report four patients with profound secondary HLH provoked by diverse triggers, including invasive pulmonary aspergillosis on background systemic lupus erythematosus, disseminated tuberculosis, and T-cell lymphoma treated with ruxolitinib as monotherapy or combination therapy in upfront and salvage settings.RESULTS:
All four patients had rapid, sustained improvement in clinical status, inflammatory markers, and hematological cell counts followed by durable remission. Three patients developed manageable infectious complications postruxolitinib.CONCLUSIONS:
This series demonstrates the effective use of JAK inhibition with ruxolitinib to control pathological immune activation in critically ill patients with secondary HLH and otherwise limited therapeutic options. JAK inhibition is also an area of urgent investigation for the treatment of cytokine storm associated with COVID-19.Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Pyrazoles
/
Protein Kinase Inhibitors
/
Lymphohistiocytosis, Hemophagocytic
/
Janus Kinases
Type of study:
Case report
/
Observational study
/
Prognostic study
Topics:
Long Covid
Limits:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
/
Young adult
Language:
English
Journal:
Eur J Haematol
Journal subject:
Hematology
Year:
2021
Document Type:
Article
Affiliation country:
Ejh.13593
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