Ruxolitinib With De-Intensified HLH-94 for the Treatment of Hemophagocytic Lymphohistiocytosis (HLH)
Status:
Not yet recruiting
Trial end date:
2027-05-31
Target enrollment:
Participant gender:
Summary
This phase II trial tests the effects of ruxolitinib in combination with a de-intensified
HLH-94 drug regimen has on patients with newly diagnosed hemophagocytic lymphohistiocytosis
(HLH), a disorder caused by dysregulated immune responses (that is, immune responses that are
too strong and cause inflammatory damage to normal tissues). The therapy used for HLH
decreases the activity of the immune system. Ruxolitinib is a type of drug called a kinase
inhibitor. It works by blocking the signals that cause inflammatory cells to multiply.
De-intensified HLH-94 is a treatment regimen that includes 4 weeks of dexamethasone with the
dose being decreased each week, and up to 4 weeks of etoposide. This combination is commonly
used to treat HLH. Dexamethasone is a steroid medication that works by fighting inflammation.
Etoposide is in a class of medications known as podophyllotoxin derivatives. It blocks a
certain enzyme needed for cell division and deoxyribonucleic acid (DNA) repair and may kill
cancer cells and is used to kill the types of white blood cells in HLH that are attacking the
body. Giving ruxolitinib in combination with a de-intensified HLH-94 drug regimen may reduce
toxic exposure to therapy while maintaining efficacy in patients with HLH.