RecruitingPhase 2Phase 3NCT05416307

Open-label Study of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (sHLH)

A Multipart, Open-label, Single-arm, Multicenter Study to Evaluate the Safety, Efficacy and Pharmacokinetics of ELA026 in Participants With Secondary Hemophagocytic Lymphohistiocytosis (sHLH)


Sponsor

Electra Therapeutics Inc.

Enrollment

156 participants

Start Date

May 19, 2022

Study Type

INTERVENTIONAL

Conditions

Summary

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.


Eligibility

Min Age: 6 Years

Inclusion Criteria11

  • ≥12 years at the time of HLH diagnosis (Cohort 1).
  • ≥6 years at the time of HLH diagnosis (Cohort 2-3).
  • 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).

Exclusion Criteria13

  • Known or previous treatment for primary HLH
  • Any other significant concurrent, uncontrolled medical condition that in the opinion of the Investigator contraindicates participation in this study
  • Unknown trigger for sHLH
  • Active, relapsed/refractory malignancy for which no suitable therapies are available to treat the malignancy triggering the HLH
  • Allogeneic hemopoietic stem cell transplant (HSCT) within 100 days of the first dose of ELA026.
  • Ongoing administration of any therapies used to treat HLH (excluding dexamethasone)
  • Live or attenuated vaccine received within 6 weeks or bacille Calmette-Guerin (BCG) vaccine within 12 weeks prior to Screening
  • Refractory sHLH (except for the safety lead-in cohort for 6-12 year olds in Cohort B).
  • Known or suspected primary or hereditary HLH.
  • Severe organ dysfunction.
  • Any other significant concurrent, uncontrolled medical condition that contraindicates participation in this study or prohibits completion of study procedures.
  • End-stage malignancy for which no suitable therapies are available to treat the malignancy triggering the HLH.
  • Allogeneic hemopoietic stem cell transplant within 100 days prior to the first dose of ELA026.

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Interventions

DRUGELA026

Multiple doses of ELA026


Locations(37)

University of Alabama at Birmingham School of Medicine

Birmingham, Alabama, United States

Phoenix Children's Hospital

Phoenix, Arizona, United States

University of California, Los Angeles

Los Angeles, California, United States

MedStar Georgetown University Hospital

Washington D.C., District of Columbia, United States

Johns Hopkins All Children's Hospital

St. Petersburg, Florida, United States

Children's Healthcare of Atlanta - Center for Advanced Pediatrics

Atlanta, Georgia, United States

Emory University

Atlanta, Georgia, United States

Columbia University

New York, New York, United States

Cornell University

New York, New York, United States

Memorial Sloan Kettering Cancer Center

New York, New York, United States

Thomas Jefferson University

Philadelphia, Pennsylvania, United States

Texas Children's Hospital

Houston, Texas, United States

University of Texas MD Anderson Cancer Center

Houston, Texas, United States

Huntsman Cancer Institute

Salt Lake City, Utah, United States

Fred Hutchinson Cancer Center - Seattle Cancer Care Alliance (SCCA) Location

Seattle, Washington, United States

Medizinische Universität Innsbruck

Innsbruck, Austria

Medical University of Vienna

Vienna, Austria

Universitätsklinikum Jena

Jena, Thuringia, Germany

Schwarzwald-Baar Klinikum Villingen-Schwenningen

Villingen-Schwenningen, Germany

Azienda Ospedaliero Universitaria Meyer

Florence, Italy

IRCCS Istituto Giannina Gaslini

Genova, Italy

Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico

Milan, Italy

Azienda Ospedale Università di Padova

Padova, Italy

Bambino Gesu' Roma

Roma, Italy

Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) - Materno-Infantile Burlo Garofolo

Trieste, Italy

Amsterdam Universitair Medische Centra - Locatie Academisch Medisch Centrum

Amsterdam, Noord-Holland - NET, Netherlands

Radboud Universitair Medisch Centrum

Nijmegen, Netherlands

Erasmus UMC

Rotterdam, Netherlands

Wilhelmina Kinderziekenhuis

Utrecht, Netherlands

Clinica Universidad de Navarra - Madrid

Madrid, Madrid - SPA, Spain

Clinica Universidad de Navarra - Pamplona

Pamplona, Navarre - SPA, Spain

Hospital Universitario Virgen del Rocio

Seville, Sevilla - SPA, Spain

Hospital Universitari i Politecnic La Fe

Valencia, Valencia - SPA, Spain

Hospital 12 de Octubre, Madrid

Madrid, Spain

Hospital Ramon y Cajal

Madrid, Spain

Hospital La Fe Valencia

Valencia, Spain

University College London Hospitals NHS Foundation Trust

London, England - UK, United Kingdom

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NCT05416307