Actively Recruiting

Phase 3
Age: 18Years +
All Genders
ID06003387

Phase 3b Open-label Study of CSL222 Gene Therapy in Adults With Severe or Moderately Severe Hemophilia B and Detectable AAV5 Neutralizing Antibodies

Led by CSL Behring · Updated on 2026-03-17

35

Participants Needed

26

Research Sites

182 weeks

Total Duration

On this page

AI-Summary

What this Trial Is About

This trial studies adults with severe or moderately severe hemophilia B who have detectable neutralizing antibodies against adeno-associated virus serotype 5 (AAV5). The purpose is to assess the risk of bleeding due to possible failure of the pharmacological action of CSL222 gene therapy in this group. CSL222 is being evaluated by CSL Behring in a phase 3b, open-label study to better understand its safety and efficacy. Participants will receive a single intravenous infusion of CSL222, delivering 2 × 10^13 genome copies per kilogram on the first day. This gene therapy aims to provide a one-time treatment targeting the underlying FIX deficiency in hemophilia B. The study does not involve placebo or multiple doses, focusing solely on the effects of this single administration. Throughout the study, participants will be closely monitored for bleeding rates between months 7 to 18 after treatment, as well as potential side effects and changes in liver function, blood counts, and Factor IX activity for up to 60 months. Assessments include laboratory tests, ultrasound scans, and patient diaries. Researchers will also evaluate quality of life and corticosteroid use. The total follow-up period allows for long-term safety and effectiveness observation.

CONDITIONS

Brief Title

Efficacy and Safety of CSL222 (Etranacogene Dezaparvovec) Gene Therapy in Adults With Hemophilia B With Pretreatment Adeno-associated Virus Serotype 5 (AAV5) Neutralizing Antibodies (Nabs)

Who Can Participate

Age: 18Years +
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Considered legally an adult as defined by local regulations
  • Diagnosed with congenital hemophilia B with severe or moderately severe Factor IX deficiency (≤ 2% normal levels)
  • On continuous routine Factor IX prophylaxis
  • Have two consecutive detectable AAV5 neutralizing antibody results between screening and final visit
  • Have more than 150 previous exposure days to Factor IX replacement therapy
  • Have been on stable Factor IX prophylaxis for at least 2 months before screening
  • Able to independently and accurately complete an electronic diary
  • Willing to follow contraception guidelines
  • Able to give informed consent and adhere to study procedures
Not Eligible

You will not qualify if you...

  • History of Factor IX inhibitors or positive inhibitor test at screening or final visit
  • Total bilirubin > 2 times upper limit of normal except if due to Gilbert's syndrome
  • Laboratory abnormalities at screening/final visit: ALT > 2x ULN, AST > 2x ULN, alkaline phosphatase > 2x ULN, serum creatinine > 2x ULN, hemoglobin < 8 g/dL
  • Any condition other than hemophilia B causing increased bleeding risk
  • Thrombocytopenia with platelet count < 50 x 10^9/L
  • Uncontrolled or untreated infections including HIV, HBV, HCV, or other significant medical conditions
  • Known allergy to corticosteroids or need for chronic oral corticosteroids
  • Uncontrolled allergic conditions or allergy to CSL222 excipients
  • Previous treatment with AAV5 gene therapy
  • Receipt of experimental agent or device within 60 days before screening until study end

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Treatment

Duration - Single day

Participants receive a single intravenous infusion of CSL222 gene therapy on Day 1.

1 infusion visit (in-person)

Follow-up

Duration - Up to 60 months after treatment

Participants are monitored for safety, efficacy, and adverse events for up to 60 months after treatment.

Regular visits during the follow-up period

Trial Site Locations

Total: 26 locations

1

University of California, San Diego (UCSD)

San Diego, California, United States, 92121

Actively Recruiting

2

University of Michigan

Ann Arbor, Michigan, United States, 48109

Actively Recruiting

3

Royal Prince Alfred Hospital

Camperdown, New South Wales, Australia, 2050

Actively Recruiting

4

Royal Brisbane Hospital

Herston, Queensland, Australia, 4029

Actively Recruiting

5

Royal Adelaide Hospital

Adelaide, South Australia, Australia, 5000

Actively Recruiting

6

The Alfred Hospital

Melbourne, Victoria, Australia, 3004

Actively Recruiting

7

McMaster University - Hamilton

Hamilton, Ontario, Canada, L8N 3Z5

Actively Recruiting

8

Queen Mary Hospital

Hong Kong, Hong Kong, 999077

Actively Recruiting

9

Prince of Wales Hospital Chinese University of Hong Kong

Shatin, Hong Kong, 999077

Actively Recruiting

10

Sheba Medical Center

Tel Litwinsky, Israel, 5265601

Actively Recruiting

11

Centro de Investigacion Clinica GRAMEL S.C.

Mexico City, Mexico City, Mexico, 3720

Actively Recruiting

12

King Faisal Specialist Hospital and Research Center

Riyadh, Saudi Arabia, 11471

Actively Recruiting

13

National University Hospital

Singapore, Singapore, 110974

Actively Recruiting

14

Singapore General Hospital

Singapore, Singapore, 169608

Actively Recruiting

15

Haemophilia Comprehensive Care Centre

Johannesburg, South Africa, 2193

Actively Recruiting

16

Kyungpook National University Hospital

Daegu, South Korea, 41944

Actively Recruiting

17

Kyung Hee University Hospital at Gangdong

Seoul, South Korea, 05278

Actively Recruiting

18

Severance Hospital, Yonsei University Health System

Seoul, South Korea, 3722

Actively Recruiting

19

Tri-Service General Hospital

Taipei, Neihu District, Taiwan, 114

Actively Recruiting

20

Kaohsiung Medical University Chung-Ho Memorial Hospital (KMUH)

Kaohsiung City, Sanmin District, Taiwan, 80756

Actively Recruiting

21

Changhua Christian Hospital (CCH)

Chang-hua, Taiwan, 500

Actively Recruiting

22

Taichung Veterans General Hospital -

Taichung, Taiwan, 40705

Actively Recruiting

23

National Taiwan University Hospital

Taipei, Taiwan, 100225

Actively Recruiting

24

Ege University Medical Faculty

Bornova, Turkey (Türkiye), 35100

Actively Recruiting

25

Gaziantep University Sahinbey Research and Practice Hospital

Gaziantep, Turkey (Türkiye), 27310

Actively Recruiting

26

Özel Acibadem Adana Hastanesi

Seyhan, Turkey (Türkiye), 01130

Actively Recruiting

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Research Team

T

Trial Registration Coordinator

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NA

Model

SINGLE_GROUP

Primary Purpose

TREATMENT

Number of Arms

1

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