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The effect of emicizumab prophylaxis on long-term, self-reported physical health in persons with haemophilia A without factor VIII inhibitors in the HAVEN 3 and HAVEN 4 studies.
Skinner, Mark W; Négrier, Claude; Paz-Priel, Ido; Chebon, Sammy; Jiménez-Yuste, Victor; Callaghan, Michael U; Lehle, Michaela; Niggli, Markus; Mahlangu, Johnny; Shapiro, Amy; Shima, Midori; Campinha-Bacote, Avrita; Levy, Gallia G; Oldenburg, Johannes; von Mackensen, Sylvia; Pipe, Steven W.
Afiliación
  • Skinner MW; Institute for Policy Advancement Ltd, Washington, District of Columbia, USA.
  • Négrier C; McMaster University, Hamilton, Canada.
  • Paz-Priel I; Louis Pradel University Hospital, Claude Bernard University Lyon 1, Lyon, France.
  • Chebon S; Genentech Inc., South San Francisco, California, USA.
  • Jiménez-Yuste V; F. Hoffmann-La Roche Ltd, Basel, Switzerland.
  • Callaghan MU; La Paz University Hospital, Autónoma University, Madrid, Spain.
  • Lehle M; Central Michigan University, Detroit, Michigan, USA.
  • Niggli M; F. Hoffmann-La Roche Ltd, Basel, Switzerland.
  • Mahlangu J; F. Hoffmann-La Roche Ltd, Basel, Switzerland.
  • Shapiro A; University of the Witwatersrand and NHLS, Johannesburg, South Africa.
  • Shima M; Indiana Hemophilia and Thrombosis Center, Indianapolis, Indiana, USA.
  • Campinha-Bacote A; Nara Medical University Hospital, Kashihara, Japan.
  • Levy GG; Genentech Inc., South San Francisco, California, USA.
  • Oldenburg J; Genentech Inc., South San Francisco, California, USA.
  • von Mackensen S; Spark Therapeutics, Inc., Philadelphia, Pennsylvania, USA.
  • Pipe SW; Universitätsklinikum Bonn, Bonn, Germany.
Haemophilia ; 27(5): 854-865, 2021 Sep.
Article en En | MEDLINE | ID: mdl-34171159
INTRODUCTION: Severe haemophilia A (HA) has a major impact on health-related quality of life (HRQoL). AIM: Assess the impact of emicizumab on HRQoL in persons with severe HA (PwHA) without factor VIII (FVIII) inhibitors in the phase 3 HAVEN 3 and 4 studies. METHODS: This pooled analysis examines the HRQoL of PwHA aged ≥ 18 years treated with emicizumab prophylaxis via Haemophilia-Specific Quality of Life Questionnaire for Adults (Haem-A-QoL) and EuroQoL 5-Dimensions 5-levels (EQ-5D-5L). In particular, changes from baseline in Haem-A-QoL 'Physical Health' (PH) domain and 'Total Score' (TS) are evaluated. RESULTS: Among 176 evaluable participants, 96 (55%) had received prior episodic treatment and 80 (45%) prophylaxis; 70% had ≥ 1 target joint and 51% had experienced ≥ 9 bleeds in the previous 24 weeks. Mean Haem-A-QoL PH and TS improved after emicizumab initiation. Mean (standard deviation) -12.0 (21.26)- and -8.6 (12.57)-point improvements were observed in PH and TS from baseline to Week 73; Week 73 scores were 27.9 (24.54) and 22.0 (14.38), respectively. Fifty-four percent of participants reported a clinically meaningful improvement in PH scores (≥ 10 points) by Week 73. Subgroups with poorer HRQoL prior to starting emicizumab (i.e. receiving episodic treatment, ≥ 9 bleeds, target joints) had the greatest improvements in PH scores, and corresponding reductions in missed workdays; change was not detected among those previously taking prophylaxis. No change over time was detected by the EQ-5D-5L questionnaire. CONCLUSIONS: Emicizumab prophylaxis in PwHA without FVIII inhibitors resulted in persistent and meaningful improvements in Haem-A-QoL PH and less work disruption than previous treatment.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Hemofilia A Aspecto: Patient_preference Límite: Adult / Humans Idioma: En Revista: Haemophilia Asunto de la revista: HEMATOLOGIA Año: 2021 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Hemofilia A Aspecto: Patient_preference Límite: Adult / Humans Idioma: En Revista: Haemophilia Asunto de la revista: HEMATOLOGIA Año: 2021 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido