Study to Assess the Efficacy and Safety of Emapalumab in Primary Haemophagocytic Lymphohistiocytosis

NCT ID: NCT03312751

Last Updated: 2024-03-12

Study Results

Results available

Outcome measurements, participant flow, baseline characteristics, and adverse events have been published for this study.

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

35 participants

Study Classification

INTERVENTIONAL

Study Start Date

2019-02-06

Study Completion Date

2022-09-14

Brief Summary

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The purpose of this study is to expand the knowledge on the efficacy and safety of emapalumab (previously known as NI-0501) as a treatment for primary haemophagocytic lymphohistiocytosis (HLH) patients, including on long-term outcomes and quality of life assessments. Emapalumab can be administered as the first-line therapy to patients not previously treated with the current standard of care, or can be given to patients who have either failed or were unable to tolerate the available standard of care.

Emapalumab is to be administered until the start of conditioning for hematopoietic stem cell transplantation (HSCT), with an anticipated duration ranging from a minimum of 4 weeks to approximately 12 weeks and not exceeding 6 months.

After treatment completion, patients will continue in the study for long-term follow-up until 1 year after either HSCT or last emapalumab infusion (if HSCT is not performed).

Detailed Description

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Conditions

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Primary Hemophagocytic Lymphohistiocytosis

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Emapalumab

Group Type EXPERIMENTAL

Emapalumab

Intervention Type DRUG

Emapalumab will be administered by intravenous infusion, twice weekly.

Interventions

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Emapalumab

Emapalumab will be administered by intravenous infusion, twice weekly.

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Primary HLH patients with active disease.
* Treatment naïve patients or patients having already received HLH conventional therapy, but having not responded, not achieved a satisfactory response or worsened, or reactivated, or are unable to tolerate current standard of care.
* Informed consent signed by the patient or by the patient's legally authorized representative.
* Received guidance on contraception.

Exclusion Criteria

* Diagnosis of secondary HLH consequent to a proven rheumatic, metabolic or neoplastic disease.
* Active mycobacteria, Histoplasma capsulatum, Shigella, Salmonella, Campylobacter or Leishmania infections.
* Evidence of latent tuberculosis.
* Presence of malignancy.
* Concomitant disease or malformation severely affecting cardiovascular, pulmonary, central nervous system (CNS), liver, or renal function, that in the opinion of the Investigator may significantly affect the likelihood to respond to treatment and/or the assessment of emapalumab safety and/or efficacy.
* History of hypersensitivity or allergy to any component of the study regimen.
* Receipt of a BCG vaccine within 12 weeks prior to Screening.
* Receipt of a live or attenuated-live (other than BCG) vaccine within 6 weeks prior to Screening.
* Pregnant or lactating female patients.
Maximum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Swedish Orphan Biovitrum

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Phoenix Children Hospital

Phoenix, Arizona, United States

Site Status

Children's Hospital Los Angeles

Los Angeles, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

Alfred I. duPont Hospital for Children - Nemours Center for Cancer and Blood Disorders - Division of Pediatric Hematology Oncology

Wilmington, Delaware, United States

Site Status

Children's Healthcare of Atlanta

Atlanta, Georgia, United States

Site Status

Dana-Farber Cancer Institute (DFCI)

Boston, Massachusetts, United States

Site Status

Spectrum Health Helen DeVos Children's Hospital

Grand Rapids, Michigan, United States

Site Status

Cincinnati Children's Hospital

Cincinnati, Ohio, United States

Site Status

Texas Children's Hospital - Feigin Center

Houston, Texas, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

Hopital Ste-Justine Research Center

Montreal, , Canada

Site Status

Hospital for Sick Children

Toronto, , Canada

Site Status

Children's and Women's Health Centre of British Columbia

Vancouver, , Canada

Site Status

Universitätsklinikum Essen

Essen, , Germany

Site Status

Medical Center- University of Freiburg

Freiburg im Breisgau, , Germany

Site Status

Universitätsklinikum Eppendorf

Hamburg, , Germany

Site Status

Istituto Giannina Gaslini

Genova, , Italy

Site Status

Fondazione MBBM, Ospedale San Gerardo

Monza, , Italy

Site Status

Ospedale Pediatrico Bambino Gesù

Rome, , Italy

Site Status

Ospedale della Donna e del Bambino

Verona, , Italy

Site Status

Hospital Universitario Vall d'Hebron

Barcelona, , Spain

Site Status

Hospital Universitario Niño Jesús

Madrid, , Spain

Site Status

Karolinska University Hospital Huddinge

Stockholm, , Sweden

Site Status

University Children's Hospital Zurich

Zurich, , Switzerland

Site Status

Leeds Children's Hospital

Leeds, , United Kingdom

Site Status

Great Ormond Street Hospital

London, , United Kingdom

Site Status

Royal Manchester Children's Hospital

Manchester, , United Kingdom

Site Status

Countries

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United States Canada Germany Italy Spain Sweden Switzerland United Kingdom

Provided Documents

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Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Other Identifiers

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NI-0501-09

Identifier Type: -

Identifier Source: org_study_id

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