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RESULTS FROM A PHASE 3, MULTICENTER, NONINFERIORITY STUDY OF RAVULIZUMAB (ALXN1210) VERSUS ECULIZUMAB IN ADULT PATIENTS WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) NAIVE TO COMPLEMENT INHIBITORS
Author(s): ,
Jong Wook Lee
Affiliations:
Department of Hematology,Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea,Seoul,Korea, Republic Of
,
Scott T. Rottinghaus
Affiliations:
Alexion Pharmaceuticals, Inc.,New Haven,United States
,
Lily Wong Lee Lee
Affiliations:
Hematology Unit,Queen Elizabeth Hospital, Kota Kinabalu,Sabah,Malaysia
,
Viviani Pessoa
Affiliations:
Hematology,Hemorio,Rio de Janeiro,Brazil
,
Sandra Gualandro
Affiliations:
Department of Haematology,University of São Paulo Medical School,São Paulo,Brazil
,
Wolfgang Füreder
Affiliations:
Department of Internal Medicine I, Division of Hematology & Hemostaseology,Medical University of Vienna,Vienna,Austria
,
Vadim Ptushkin
Affiliations:
Outpatient Department for Hematology, Oncology and Chemotherapy,S.P. Botkin Hospital,Moscow,Russian Federation
,
Flore Sicre de Fontbrune
Affiliations:
Hôpital Saint-Louis,Paris,France
,
Lori Volles
Affiliations:
Alexion Pharmaceuticals, Inc.,New Haven,United States
,
Lori Shafner
Affiliations:
Alexion Pharmaceuticals, Inc.,New Haven,United States
,
Andrew I. Damokosh
Affiliations:
Alexion Pharmaceuticals, Inc.,New Haven,United States
,
Rasha Aguzzi
Affiliations:
Alexion Pharmaceuticals, Inc.,Lexington,United States
,
Rajendra Pradhan
Affiliations:
Alexion Pharmaceuticals, Inc.,New Haven,United States
,
Stephan Ortiz
Affiliations:
Alexion Pharmaceuticals, Inc.,New Haven,United States
,
Hubert Schrezenmeier
Affiliations:
Institute of Transfusion Medicine, University of Ulm, and Institute for Clinical Transfusion Medicine and Immunogenetics, German Red Cross Blood Transfusion Service Baden-Württemberg-Hessen and University Hospital Ulm,Ulm,Germany
Anita Hill
Affiliations:
Department of Haematology,Leeds Teaching Hospitals,Leeds,United Kingdom
(Abstract release date: 06/01/18) EHA Library. Lee J. 06/17/18; 218885; LB2603
Prof. Dr. Jong Wook Lee
Prof. Dr. Jong Wook Lee
Contributions
Abstract

Abstract: LB2603

Type: Oral Presentation

Presentation during EHA23: On Sunday, June 17, 2018 from 11:45 - 12:00

Location: Room A1

Background
Ravulizumab, an innovative C5 inhibitor with high C5 affinity and half-life 3-4 times longer than eculizumab, provides immediate, complete, and sustained C5 inhibition with extended dosing intervals.

Aims
To compare efficacy and safety of ravulizumab vs eculizumab in adult patients (pts) with PNH naive to complement inhibitor therapy (NCT02946463).

Methods
In this phase 3, randomized, open-label, noninferiority, multicenter study, complement inhibitor-naive PNH pts with ≥1 PNH-related sign/symptom and LDH level ≥1.5 xULN at screening were stratified by transfusion history (0, 1-14, or >14 units of red blood cells 1 yr before study) and screening LDH level (1.5-<3 xULN or ≥3 xULN), and randomized 1:1 to receive ravulizumab (loading/maintenance dose [day 1/day 15 and q8w thereafter] as follows: ≥40-<60 kg body weight [2400/3000 mg]; ≥60-<100 kg [2700/3300 mg]; ≥100 kg [3000/3600 mg]), or eculizumab at approved dosing regimen through day 183 (primary evaluation period). Primary efficacy endpoints included transfusion avoidance (TA; proportion of pts who remain transfusion-free) and LDH normalization (LDH ULN=246 U/L) from days 29–183. Noninferiority was declared if the lower bound of the 95% CI for difference in proportion of pts with TA receiving ravulizumab vs eculizumab was >–20% and lower bound of the 95% CI for odds ratio for LDH normalization between ravulizumab vs eculizumab was >0.39. Key secondary endpoints were % change in LDH from baseline (BL), change from BL in FACIT-Fatigue score, and proportions of pts with breakthrough hemolysis (BTH) and stabilized hemoglobin (Hb). Pharmacodynamic effects were measured by change in free C5.

Results
Of 285 pts screened, 246 from countries across the Asia-Pacific region, Europe, and North and South America were randomized. All 125 ravulizumab pts and 119/121 eculizumab pts completed 26 wks of treatment. Ravulizumab met the primary objective of statistically significant noninferiority vs eculizumab on both primary endpoints and all 4 key secondary endpoints, with trends favoring ravulizumab over eculizumab for all 6 endpoints. Ravulizumab was noninferior to eculizumab in proportions of pts achieving TA (73.6% vs 66.1%; difference, 6.8% [95% CI, -4.7, 18.1%]) and LDH normalization (53.6% vs 49.4%, OR 1.19 [0.80, 1.77]) (Fig A&C). Point estimates favored ravulizumab in % change in least squares mean LDH levels (76.8% vs 76.0% reduction from BL), change in FACIT-Fatigue score (7.1 vs 6.4 point improvement from BL), and % of pts with BTH (4.0% vs 10.7%) and Hb stabilization (68.0% vs 64.5%) (Fig B&C). Immediate, complete, and sustained inhibition of C5 (mean free C5 <0.5 μg/mL) was observed by end of first infusion of ravulizumab (Fig D). Most frequently reported treatment-emergent adverse event (AE) was headache (36.0%/33.1% in pts receiving ravulizumab/eculizumab). Serious AEs were experienced by 8.8%/7.4% in the ravulizumab/eculizumab groups. Major adverse vascular events occurred in 3 pts (ravulizumab, 2; eculizumab, 1). There were no meningococcal infections.

Conclusion
In the largest study of PNH pts, q8w ravulizumab achieved statistically significant noninferiority to q2w eculizumab on all primary and key secondary endpoints, with an efficacy profile consistent with immediate, complete, and sustained inhibition of free C5. Safety was similar in both groups.

Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical

Keyword(s): Complement, Monoclonal antibody, transfusion, Paroxysmal nocturnal hemoglobinuria (PNH)

Abstract: LB2603

Type: Oral Presentation

Presentation during EHA23: On Sunday, June 17, 2018 from 11:45 - 12:00

Location: Room A1

Background
Ravulizumab, an innovative C5 inhibitor with high C5 affinity and half-life 3-4 times longer than eculizumab, provides immediate, complete, and sustained C5 inhibition with extended dosing intervals.

Aims
To compare efficacy and safety of ravulizumab vs eculizumab in adult patients (pts) with PNH naive to complement inhibitor therapy (NCT02946463).

Methods
In this phase 3, randomized, open-label, noninferiority, multicenter study, complement inhibitor-naive PNH pts with ≥1 PNH-related sign/symptom and LDH level ≥1.5 xULN at screening were stratified by transfusion history (0, 1-14, or >14 units of red blood cells 1 yr before study) and screening LDH level (1.5-<3 xULN or ≥3 xULN), and randomized 1:1 to receive ravulizumab (loading/maintenance dose [day 1/day 15 and q8w thereafter] as follows: ≥40-<60 kg body weight [2400/3000 mg]; ≥60-<100 kg [2700/3300 mg]; ≥100 kg [3000/3600 mg]), or eculizumab at approved dosing regimen through day 183 (primary evaluation period). Primary efficacy endpoints included transfusion avoidance (TA; proportion of pts who remain transfusion-free) and LDH normalization (LDH ULN=246 U/L) from days 29–183. Noninferiority was declared if the lower bound of the 95% CI for difference in proportion of pts with TA receiving ravulizumab vs eculizumab was >–20% and lower bound of the 95% CI for odds ratio for LDH normalization between ravulizumab vs eculizumab was >0.39. Key secondary endpoints were % change in LDH from baseline (BL), change from BL in FACIT-Fatigue score, and proportions of pts with breakthrough hemolysis (BTH) and stabilized hemoglobin (Hb). Pharmacodynamic effects were measured by change in free C5.

Results
Of 285 pts screened, 246 from countries across the Asia-Pacific region, Europe, and North and South America were randomized. All 125 ravulizumab pts and 119/121 eculizumab pts completed 26 wks of treatment. Ravulizumab met the primary objective of statistically significant noninferiority vs eculizumab on both primary endpoints and all 4 key secondary endpoints, with trends favoring ravulizumab over eculizumab for all 6 endpoints. Ravulizumab was noninferior to eculizumab in proportions of pts achieving TA (73.6% vs 66.1%; difference, 6.8% [95% CI, -4.7, 18.1%]) and LDH normalization (53.6% vs 49.4%, OR 1.19 [0.80, 1.77]) (Fig A&C). Point estimates favored ravulizumab in % change in least squares mean LDH levels (76.8% vs 76.0% reduction from BL), change in FACIT-Fatigue score (7.1 vs 6.4 point improvement from BL), and % of pts with BTH (4.0% vs 10.7%) and Hb stabilization (68.0% vs 64.5%) (Fig B&C). Immediate, complete, and sustained inhibition of C5 (mean free C5 <0.5 μg/mL) was observed by end of first infusion of ravulizumab (Fig D). Most frequently reported treatment-emergent adverse event (AE) was headache (36.0%/33.1% in pts receiving ravulizumab/eculizumab). Serious AEs were experienced by 8.8%/7.4% in the ravulizumab/eculizumab groups. Major adverse vascular events occurred in 3 pts (ravulizumab, 2; eculizumab, 1). There were no meningococcal infections.

Conclusion
In the largest study of PNH pts, q8w ravulizumab achieved statistically significant noninferiority to q2w eculizumab on all primary and key secondary endpoints, with an efficacy profile consistent with immediate, complete, and sustained inhibition of free C5. Safety was similar in both groups.

Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical

Keyword(s): Complement, Monoclonal antibody, transfusion, Paroxysmal nocturnal hemoglobinuria (PNH)

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