A Multipart, Open-label, Single-arm, Multicenter Study to Evaluate the Safety, Efficacy and Pharmacokinetics of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (sHLH)
Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome of excessive immune activation. Secondary hemophagocytic lymphohistiocytosis (sHLH) is the most common form of this disease and is typically associated with several other clinical conditions (eg, malignancy associated HLH (mHLH), infection, or autoimmune disease). ELA026 is a fully human immunoglobulin G1 (IgG1) signal regulatory protein (SIRP)-directed monoclonal antibody designed to deplete the myeloid and T cells driving the inflammation. The purpose of this study is to assess the safety, efficacy pharmacokinetics and pharmacodynamics of ELA026 in participants with sHLH.
• ≥12 years at the time of HLH diagnosis (Cohort 1).
• Treatment naïve or relapsed/refractory (Cohorts 1 and 2).
• Treatment naïve or early refractory (Cohort 3).
• Participant with sHLH confirmed criteria based on fulfilling 5 out of 8 HLH-2004 diagnostic criteria.
• Cohort A: Adults with newly diagnosed, treatment-naïve, malignancy-associated sHLH.
• Cohort B: Adults with newly diagnosed, treatment-naïve, non-malignancy-associated sHLH.
• Cohort B: Adults with newly diagnosed, treatment-naïve, malignancy-associated sHLH, diagnosed by OHI index.
• Cohort B: 13 to 17 years olds with newly diagnosed, treatment-naïve sHLH.
• Cohort B: 6 to 12 year olds, with refractory sHLH (safety lead-in cohort).
• Cohort B: 6 to 12 year olds, with newly diagnosed, treatment-naïve sHLH (after completion of safety lead-in cohort).