Danicopan as Add-on Therapy to a C5 Inhibitor in Paroxysmal Nocturnal Hemoglobinuria (PNH) Participants Who Have Clinically Evident Extravascular Hemolysis (EVH)(ALPHA)

NCT ID: NCT04469465

Last Updated: 2025-05-04

Study Results

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

86 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-12-16

Study Completion Date

2024-01-16

Brief Summary

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The main objective of this study is to evaluate the efficacy of danicopan as add-on therapy to a complement component 5 (C5) inhibitor (eculizumab or ravulizumab) in participants with PNH who have clinically evident EVH.

Detailed Description

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This is a multiple-region, randomized, double-blind, placebo controlled, multiple-dose, study in participants with PNH who have clinically evident EVH on a C5 inhibitor (eculizumab or ravulizumab).

Participants will be randomized to receive danicopan or placebo, in a 2:1 ratio for 12 weeks (Treatment Period 1) in addition to their C5 inhibitor (eculizumab or ravulizumab) therapy. At Week 12, participants randomized to receive placebo will be switched to danicopan in addition to their C5 inhibitor for an additional 12 weeks (Treatment Period 2) and participants randomized to danicopan will continue on danicopan for an additional 12 weeks, while remaining on their ongoing C5 inhibitor therapy.

At the end of the 2 treatment periods (Week 24), participants may enter a Long-Term Extension (LTE) Period and continue to receive danicopan in addition to their C5 inhibitor therapy. The Long-Term Extension period will consist of a first year of LTE(Year1) and a second year of optional LTE(Year2).All patients will complete 72 weeks of LTE(Year 1) assessments. After Week 72 (at the end of the first year of LTE), patients have the choice to complete participation in this study or continue to the optional second year (Year2) of LTE.

Conditions

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Paroxysmal Nocturnal Hemoglobinuria

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

DOUBLE

Participants Investigators

Study Groups

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Danicopan + C5 Inhibitor

Participants will receive danicopan, in addition to their C5 inhibitor therapy, for 24 weeks (12 weeks in Treatment Period 1, followed by 12 weeks in Treatment Period 2).

Group Type EXPERIMENTAL

Danicopan

Intervention Type DRUG

Oral tablet

C5 Inhibitor

Intervention Type DRUG

Participants will continue to receive their ongoing C5 inhibitor (eculizumab or ravulizumab) therapy according to their usual dose and schedule.

Placebo + C5 Inhibitor

Participants will receive placebo, in addition to their C5 inhibitor therapy, for 12 weeks during Treatment Period 1. At Week 12, participants randomized to receive placebo will be switched to danicopan for an additional 12 weeks (Treatment Period 2).

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Oral tablet

C5 Inhibitor

Intervention Type DRUG

Participants will continue to receive their ongoing C5 inhibitor (eculizumab or ravulizumab) therapy according to their usual dose and schedule.

Interventions

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Danicopan

Oral tablet

Intervention Type DRUG

Placebo

Oral tablet

Intervention Type DRUG

C5 Inhibitor

Participants will continue to receive their ongoing C5 inhibitor (eculizumab or ravulizumab) therapy according to their usual dose and schedule.

Intervention Type DRUG

Other Intervention Names

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ALXN2040

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of PNH
* Clinically Evident EVH defined by:

* Anemia (Hgb ≤9.5 gram/deciliter) with absolute reticulocyte count ≥120 x 10\^9/liter
* Receiving an approved C5 inhibitor for at least 6 months prior to Day 1
* Platelet count ≥30,000/microliters (µL)
* Absolute neutrophil counts ≥500/μL
* Documentation of/or willingness to receive vaccinations for N. meningiditis and prophylactic antibiotics as required

Exclusion Criteria

* History of a major organ transplant or hematopoietic stem cell transplantation (HSCT)
* Participants with known aplastic anemia or other bone marrow failure that requires HSCT or other therapies including anti-thymocyte globulin and/or immunosuppressants
* Known or suspected complement deficiency
* Laboratory abnormalities at screening, including:

* Alanine aminotransferase \>2 x ULN (\>3 x ULN in case of patients with documented liver iron overload defined by serum ferratin values

* 500 ng/ML)
* Direct bilirubin \>2 x ULN (unless due to EVH or documented Gilbert's Syndrome)
* Current evidence of biliary cholestasis
* Estimated glomerular filtration rate of \<30 milliliters/minute/1.73 meter squared and/or are on dialysis
* Evidence of human immunodeficiency virus, hepatitis B, or active hepatitis C infection at screening
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Alexion Pharmaceuticals, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Research Site

Los Angeles, California, United States

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Weston, Florida, United States

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Chicago, Illinois, United States

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Kalamazoo, Michigan, United States

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New York, New York, United States

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Dallas, Texas, United States

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Milwaukee, Wisconsin, United States

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Belém, , Brazil

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Curitiba, , Brazil

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Goiânia, , Brazil

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Porto Alegre, , Brazil

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Rio de de Janeiro, , Brazil

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Toronto, Ontario, Canada

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Brno, , Czechia

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Lille, , France

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Paris, , France

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Pessac, , France

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Pierre-Bénite, , France

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Ulm, , Germany

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Athens, , Greece

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Thessaloniki, , Greece

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Haifa, , Israel

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Jerusalem, , Israel

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Avellino, , Italy

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Bassano del Grappa, , Italy

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Florence, , Italy

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Milan, , Italy

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Reggio Calabria, , Italy

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Roma, , Italy

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Bunkyō City, , Japan

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Fukuoka, , Japan

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Kashiwa-shi, , Japan

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Kyoto, , Japan

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Nagakute-shi, , Japan

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Ogaki-shi, , Japan

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Osaka, , Japan

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Sayama, , Japan

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Shibuya-ku, , Japan

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Tanabe-shi, , Japan

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Toyoake-shi, , Japan

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Tsukuba, , Japan

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Kota Kinabalu, , Malaysia

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Kuching, , Malaysia

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Miri, , Malaysia

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Maastricht, , Netherlands

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Gdansk, , Poland

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Daejeon, , South Korea

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Seoul, , South Korea

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Seoul, , South Korea

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Seoul, , South Korea

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Suwon, , South Korea

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Barcelona, , Spain

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Barcelona, , Spain

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Las Palmas de Gran Canaria, , Spain

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Madrid, , Spain

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Majadahonda, , Spain

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Seville, , Spain

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Taipei, , Taiwan

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Bangkok, , Thailand

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Airdrie, , United Kingdom

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Leeds, , United Kingdom

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London, , United Kingdom

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Countries

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United States Brazil Canada Czechia France Germany Greece Israel Italy Japan Malaysia Netherlands Poland South Korea Spain Taiwan Thailand United Kingdom

References

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Lee JW, Griffin M, Kim JS, Lee Lee LW, Piatek C, Nishimura JI, Carrillo Infante C, Jain D, Liu P, Filippov G, Sicre de Fontbrune F, Risitano A, Kulasekararaj AG; ALXN2040-PNH-301 Investigators. Addition of danicopan to ravulizumab or eculizumab in patients with paroxysmal nocturnal haemoglobinuria and clinically significant extravascular haemolysis (ALPHA): a double-blind, randomised, phase 3 trial. Lancet Haematol. 2023 Dec;10(12):e955-e965. doi: 10.1016/S2352-3026(23)00315-0.

Reference Type RESULT
PMID: 38030318 (View on PubMed)

Kulasekararaj A, Griffin M, Piatek C, Shammo J, Nishimura JI, Patriquin C, Schrezenmeier H, Barcellini W, Panse J, Gaya A, Patel Y, Liu P, Filippov G, Sicre de Fontbrune F, Risitano A, Lee JW. Long-term efficacy and safety of danicopan as add-on therapy to ravulizumab or eculizumab in PNH with significant EVH. Blood. 2025 Feb 20;145(8):811-822. doi: 10.1182/blood.2024026299.

Reference Type DERIVED
PMID: 39700502 (View on PubMed)

Provided Documents

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Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Related Links

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Other Identifiers

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2019-003829-18

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

ALXN2040-PNH-301

Identifier Type: -

Identifier Source: org_study_id

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