Background
HLH
LYMPHOHISTIOCYTOSIS, DISORDER
Edited by: Randy Q. Cron, University of Alabama at Birmingham, United StatesReviewed by: Melissa Hines, St. Jude Children’s Research Hospital, United States; Hongsheng Zhou, Southern Medical University, China†These authors have contributed equally to this workHemophagocytic lymphohistiocytosis (HLH) is a severe disorder characterized by excessive secretion of cytokines. Even with the recommended HLH-94/2004 regimen, over 30% of patients remain refractory to frontline therapy or relapse after an initial response, leading to poor clinical outcomes. Ruxolitinib, a JAK1/2 inhibitor targets key cytokines in HLH, has shown promising therapeutic effects. However, there has been little attention given to patients who do not respond to ruxolitinib and whether an escalating dose can provide a resolution.