Treatment response and neurofilament light chain levels with long-term patisiran in hereditary transthyretin-mediated amyloidosis with polyneuropathy: 24-month results of an open-label extension study.

ATTRv amyloidosis hATTR amyloidosis neurofilament light chain patisiran polyneuropathy

Journal

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
ISSN: 1744-2818
Titre abrégé: Amyloid
Pays: England
ID NLM: 9433802

Informations de publication

Date de publication:
20 Jul 2023
Historique:
pubmed: 20 7 2023
medline: 20 7 2023
entrez: 20 7 2023
Statut: aheadofprint

Résumé

Longitudinal changes in neurofilament light chain (NfL) levels were evaluated alongside prespecified clinical assessments 24 months into the patisiran Global open-label extension (OLE) study in patients with ATTRv amyloidosis with polyneuropathy. All patients enrolled in the Global OLE, from phase III APOLLO and phase II OLE parent studies, received patisiran. Assessments included measures of polyneuropathy (modified Neuropathy Impairment Score+7 (mNIS+7)), quality of life (QOL; Norfolk QOL-Diabetic Neuropathy questionnaire (Norfolk QOL-DN)), and plasma NfL. Patients receiving patisiran in the parent study (APOLLO-patisiran, Long-term patisiran treatment led to sustained improvements in neuropathy and QOL, with NfL demonstrating potential as a biomarker for disease progression and treatment response in ATTRv amyloidosis with polyneuropathy.

Sections du résumé

BACKGROUND UNASSIGNED
Longitudinal changes in neurofilament light chain (NfL) levels were evaluated alongside prespecified clinical assessments 24 months into the patisiran Global open-label extension (OLE) study in patients with ATTRv amyloidosis with polyneuropathy.
METHODS UNASSIGNED
All patients enrolled in the Global OLE, from phase III APOLLO and phase II OLE parent studies, received patisiran. Assessments included measures of polyneuropathy (modified Neuropathy Impairment Score+7 (mNIS+7)), quality of life (QOL; Norfolk QOL-Diabetic Neuropathy questionnaire (Norfolk QOL-DN)), and plasma NfL.
RESULTS UNASSIGNED
Patients receiving patisiran in the parent study (APOLLO-patisiran,
CONCLUSIONS UNASSIGNED
Long-term patisiran treatment led to sustained improvements in neuropathy and QOL, with NfL demonstrating potential as a biomarker for disease progression and treatment response in ATTRv amyloidosis with polyneuropathy.

Identifiants

pubmed: 37469249
doi: 10.1080/13506129.2023.2232520
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1-11

Commentaires et corrections

Type : ErratumIn

Auteurs

Simina Ticau (S)

Alnylam Pharmaceuticals, Cambridge, MA, USA.

Emre Aldinc (E)

Alnylam Pharmaceuticals, Cambridge, MA, USA.

Michael Polydefkis (M)

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

David Adams (D)

Neurology Department, AP-HP, CHU Bicêtre, Université Paris-Saclay, Paris-Saclay, France.

Teresa Coelho (T)

Centro Hospitalar Universitário Santo António, European Reference Network - EUroNMD, Porto, Portugal.

Mitsuharu Ueda (M)

Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.

Cecilia Hale (C)

Alnylam Pharmaceuticals, Cambridge, MA, USA.

John Vest (J)

Alnylam Pharmaceuticals, Cambridge, MA, USA.

Paul Nioi (P)

Alnylam Pharmaceuticals, Cambridge, MA, USA.

Classifications MeSH