ELA026 / Electra Therap - LARVOL DELTA

The surpass trial: a phase 2/3 registrational trial of ELA026 in subjects with treatment-Naïve secondary hemophagocytic lymphohistiocytosis (ASH 2025) - P2/3 | "Enrollment is anticipated to begin in 2025. Studyresults will support the registration of ELA026 in sHLH."

Clinical • P2/3 data • Hematological Malignancies • Hemophagocytic lymphohistiocytosis • Immunology • Infectious Disease • Lymphoma • Rare Diseases • Rheumatology

Signal regulating proteins (SIRPs) participate in sarcoidosis granuloma formation by promoting immune cell aggregation. (PubMed, Respir Res) - "SIRPα is robustly expressed in monocyte/macrophage lineages within human sarcoidosis tissues, and inhibition of SIRPs (ELA026) suppresses sarcoidosis granuloma formation by preventing macrophage adhesion/aggregation."

Journal • Immunology • Lung Cancer • Oncology • Pulmonary Disease • Respiratory Diseases • Sarcoidosis • Solid Tumor • IL1B • SIRPA • TNFA

A Phase 1b Study of ELA026, a Monoclonal Antibody Targeting Signal Regulatory Protein-α/β1/γ, in Patients with Newly Diagnosed and Previously Treated Secondary Hemophagocytic Lymphohistiocytosis (ASH 2023) - P2/3 | "Three patients were refractory to etoposide-based therapies and/or anti-cytokine therapies. Preliminary data suggest ELA026 is well tolerated in sHLH patients and induces early biomarker changes that warrant further clinical investigation."

Clinical • P1 data • Hemophagocytic lymphohistiocytosis • Immunology • Infectious Disease • Rare Diseases • CRP

ELA026, a Monoclonal Antibody Targeting Signal Regulatory Protein-α/β1/γ, Rapidly Controls Inflammation and Improves 2-Month Survival in Treatment-Naïve Malignancy-Associated Hemophagocytic Lymphohistiocytosis (ASH 2024) - P2/3 | "Cytotoxic, multi-agent chemotherapy (including etoposide) does not address the poor prognosis associated with mHLH (Xie et al...All patients receive dexamethasone as background therapy and premedication initially...Conclusion : Data suggest ELA026 is well-tolerated to date in sHLH patients and results in a 100% response rate and improved 2-month survival in treatment-naïve mHLH patients. The study is enrolling in an expanded cohort to further assess efficacy in this patient population."

Diffuse Large B Cell Lymphoma • Hematological Malignancies • Hemophagocytic lymphohistiocytosis • Hepatology • Hodgkin Lymphoma • Immunology • Infectious Disease • Liver Failure • Lymphoma • Oncology • Pediatrics • Rare Diseases • T Cell Non-Hodgkin Lymphoma • CRP • SIRPA

Electra Therapeutics Announces $183 Million Series C Financing to Advance First-in-Class SIRP-Targeted Therapies for Immune Disorders… (GlobeNewswire) - "Proceeds from the financing will fund a global pivotal Phase 2/3 study of ELA026 in secondary hemophagocytic lymphohistiocytosis (sHLH), a hyperinflammatory disease with high mortality and a lack of treatment options. The pivotal study is enrolling patients at research sites across the U.S. and Europe and has initiated dosing....Financing will also...advance second pipeline program, ELA822, into the clinic for diseases in immunology and inflammation (I&I)."

Financing • Trial status • Hemophagocytic lymphohistiocytosis

Electra Therapeutics Announces $183 Million Series C Financing to Advance First-in-Class SIRP-Targeted Therapies for…Cancer (GlobeNewswire) - "Financing will also support indication expansion of ELA026 in hematologic cancers..."

Financing • Hematological Malignancies

Electra Therapeutics Receives FDA Breakthrough Therapy and EMA Priority Medicines Designations for ELA026 in Secondary Hemophagocytic Lymphohistiocytosis (sHLH) (The Manila Times) - "The BTD and PRIME designations are supported by positive results from the completed Phase 1b study of ELA026 in sHLH."

Breakthrough therapy • European regulatory • Hemophagocytic lymphohistiocytosis

ELA026 CP002: Open-label Study of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (sHLH) (clinicaltrials.gov) - P2/3 | N=156 | Recruiting | Sponsor: Electra Therapeutics Inc. | Active, not recruiting ➔ Recruiting

Enrollment open • Hemophagocytic lymphohistiocytosis • Immunology • Rare Diseases

ELA026 CP002: Open-label Study of ELA026 in Participants with Secondary Hemophagocytic Lymphohistiocytosis (sHLH) (clinicaltrials.gov) - P2/3 | N=156 | Active, not recruiting | Sponsor: Electra Therapeutics Inc. | Recruiting ➔ Active, not recruiting | Phase classification: P1 ➔ P2/3 | N=24 ➔ 156 | Trial completion date: Mar 2025 ➔ Jun 2029 | Trial primary completion date: Dec 2024 ➔ Jun 2027

Enrollment change • Enrollment closed • Phase classification • Trial completion date • Trial primary completion date • Hemophagocytic lymphohistiocytosis • Immunology • Rare Diseases

Electra Therapeutics Receives FDA Fast Track Designation for ELA026 in Secondary Hemophagocytic Lymphohistiocytosis (sHLH) (Businesswire) - "Electra Therapeutics, Inc...announced that the U.S. Food and Drug Administration (FDA) has granted Fast Track designation for ELA026 for the treatment of secondary hemophagocytic lymphohistiocytosis (sHLH). ELA026, a first-in-class antibody therapy targeting signal regulatory proteins (SIRP), is a novel approach to treat secondary HLH (sHLH), a rare, life-threatening disease for which there is no approved treatment."

Fast track • Hemophagocytic lymphohistiocytosis • Immunology

Electra Therapeutics Receives FDA Orphan Drug Designation for ELA026 for the Treatment of Hemophagocytic Lymphohistiocytosis (HLH) (Businesswire) - "Electra Therapeutics, Inc...announced that the U.S. Food and Drug Administration (FDA) has granted Orphan Drug Designation (ODD) to ELA026 for the treatment of hemophagocytic lymphohistiocytosis (HLH)."

Orphan drug • Hemophagocytic lymphohistiocytosis • Immunology

ELA026 CP002: Evaluation of the Safety and Efficacy of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (clinicaltrials.gov) - P1 | N=24 | Recruiting | Sponsor: Electra Therapeutics Inc. | Phase classification: P1b ➔ P1 | Trial completion date: Jun 2024 ➔ Mar 2025 | Trial primary completion date: Mar 2024 ➔ Dec 2024

Phase classification • Trial completion date • Trial primary completion date • Immunology • Rare Diseases

ELA026 TARGETING OF SIRP(+) IMMUNE CELLS RESULTS IN A HIGH RESPONSE RATE AND IMPROVED 2-MONTH SURVIVAL OF TREATMENT-NAIVE MALIGNANCY-ASSOCIATED HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN A PHASE 1 STUDY (EHA 2024) - P1b | "Preliminary data suggest ELA026 is well-tolerated to date in sHLH patients and results in a high response rateand improved 2-month survival in treatment-naïve mHLH patients. The study is ongoing to further assessefficacy in this patient population with no effective or approved therapie"

Immune cell • Late-breaking abstract • P1 data • Diffuse Large B Cell Lymphoma • Epstein-Barr Virus Infections • Hematological Disorders • Hematological Malignancies • Immunology • Infectious Disease • Leukemia • Lymphoma • Oncology • Pediatrics • Rare Diseases • T Cell Non-Hodgkin Lymphoma • CRP • SIRPA

Evaluation of the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of ELA026 in Single and Multiple Doses in Healthy Volunteers (clinicaltrials.gov) - P1 | N=91 | Completed | Sponsor: Electra Therapeutics Inc. | Recruiting ➔ Completed | Trial completion date: Apr 2024 ➔ Nov 2023 | Trial primary completion date: Apr 2024 ➔ Nov 2023

Trial completion • Trial completion date • Trial primary completion date

Electra Therapeutics to Present Clinical Data for ELA026 at the American Society of Hematology (ASH) 2023 Annual Meeting (Businesswire) - "Electra Therapeutics, Inc...announced today that the company will present the initial clinical data for its lead drug candidate, ELA026, in a poster at the American Society of Hematology (ASH) annual meeting being held in San Diego, California, December 9-12, 2023....ELA026 is a monoclonal antibody that targets signal regulatory protein-α/β1/γ (SIRP) to enable precise depletion of pathological immune cells. ELA026 is currently in a Phase 1b clinical study in patients with secondary hemophagocytic lymphohistiocytosis (sHLH), a life-threatening inflammatory disease."

P1 data • Immunology • Inflammation

Evaluation of the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of ELA026 in Single and Multiple Doses in Healthy Volunteers (clinicaltrials.gov) - P1 | N=120 | Recruiting | Sponsor: Electra Therapeutics Inc. | Trial completion date: Apr 2023 ➔ Apr 2024 | Trial primary completion date: Apr 2023 ➔ Apr 2024

Trial completion date • Trial primary completion date

Evaluation of the Safety and Efficacy of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (clinicaltrials.gov) - P1b | N=24 | Recruiting | Sponsor: Electra Therapeutics Inc. | Trial completion date: Dec 2023 ➔ Jun 2024 | Trial primary completion date: Sep 2023 ➔ Mar 2024

Trial completion date • Trial primary completion date • Immunology • Rare Diseases

UPDATE ON TRIAL IN PROGRESS: A PHASE 1B STUDY OF ELA026 IN PATIENTS WITH SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (SHLH) (EHA 2023) - P1b | "At the completion of Phase 1b, an appropriate dose, route of administration, and dose interval will be determined for a Phase 2/3 trial."

Clinical • IO biomarker • P1 data • Bone Marrow Transplantation • Hematological Disorders • Immunology • Pediatrics • Rare Diseases • Transplantation • CD8 • IFNG • SIRPA

Targeting Signal Regulatory Proteins (SIRPs) to Suppress Sarcoidosis Granuloma Responses (ATS 2023) - "Dose-dependent inhibition of in vitro granuloma formation by the anti-SIRP antibody, ELA026, provides novel insight into disease pathogenesis and implies a novel therapeutic target for treatment of sarcoidosis."

Immunology • Infectious Disease • Pulmonary Disease • Respiratory Diseases • Sarcoidosis • Tuberculosis • SIRPA

Evaluation of the Safety and Efficacy of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (clinicaltrials.gov) - P1b | N=24 | Recruiting | Sponsor: Electra Therapeutics Inc. | Trial completion date: Aug 2023 ➔ Dec 2023

Trial completion date • Immunology • Rare Diseases

Trial in Progress: A Phase 1b Study of ELA026 in Patients with Secondary Hemophagocytic Lymphohistiocytosis (ASH 2022) - P1b | "The HLH-94 treatment protocol (etoposide, dexamethasone, and cyclosporin) is used with variable degrees of effectiveness. Figure 1. Study Schema"

Clinical • P1 data • Bone Marrow Transplantation • Hematological Disorders • Immunology • Infectious Disease • Inflammation • Oncology • Rare Diseases • Systemic Inflammatory Response Syndrome • Transplantation • CD8 • IFNG • SIRPA

Characterization of ELA026, a Clinical-Stage Monoclonal Antibody That Rapidly and Potently Depletes Myeloid Cells and T Lymphocytes (ASH 2022) - P1b | "In vitro pharmacological studies demonstrate that ELA026 binds to SIRP proteins on the surface of primary human monocytes and T cells, inducing potent ADCC and ADCP. Consistent with these in vitro observations, in vivo administration of ELA026 in cynomolgus macaques shows a rapid and potent depletion of SIRP expressing monocytes and lymphocytes with a well-defined PK/PD relationship consistent with in vitro pharmacological results identifying ELA026 mechanisms of action. Reversibility of the PD effect was achieved following washout suggesting that ELA026 treatment is not associated with long-term immunosuppression observed with other similar cell depleting agents."

Clinical • Hematological Disorders • Immunology • Infectious Disease • Inflammation • Oncology • Rare Diseases • Systemic Inflammatory Response Syndrome • ACACA • CD8 • IFNG • SIRPA

Evaluation of the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of ELA026 in Single and Multiple Doses in Healthy Volunteers (clinicaltrials.gov) - P1 | N=120 | Recruiting | Sponsor: Electra Therapeutics Inc.

New P1 trial

Evaluation of the Safety and Efficacy of ELA026 in Patients With Secondary Hemophagocytic Lymphohistiocytosis (clinicaltrials.gov) - P1b | N=24 | Recruiting | Sponsor: Electra Therapeutics Inc.

New P1 trial • Immunology • Rare Diseases