MedPath

European VOD Registry

Completed
Conditions
Hepatic Veno-Occlusive Disease
Registration Number
NCT03032016
Lead Sponsor
Jazz Pharmaceuticals
Brief Summary

Following the licencing of a new drug, Defitelio®, indicated for the treatment of severe Veno-Occlusive Disease of the liver (sVOD), a rare but serious complication of haematopoietic stem cell transplantation (HSCT), as a specific obligation (SOB), the manufacture and marketing Authorisation Holder (MHA) (Gentium, a Jazz Pharmaceuticals Company) was required by PRAC (Pharmacovigilance Risk Assessment Committee) to set up a disease registry to collect safety and outcome data, and to assess patterns of utilization of Defitelio® in the post-approval setting. This registry is a Post Authorization Safety Study (PASS), is being coordinated in collaboration with the European Society for Blood and Marrow Transplantation (EBMT). For this study, anonymised clinical data are being collected from patients who develop VOD and and treated with and patients who have been treated with Defitelio® for conditions other than sVOD.

The study DOES NOT involve decisions about treatment, which are clinical decisions, but merely collection of data for patients who develop this complication, whether or not they receive treatment and for patients who are treated with Defitelio® for any other reasons.The study DOES NOT involve decisions about treatment, which are clinical decisions, but merely collection of data for patients who develop this complication, whether or not they receive treatment and for patients who are treated with Defitelio® for any other reasons.

Detailed Description

Defitelio® was granted a Marketing Authorisation in Europe under exceptional circumstances on 18 October 2013. Defitelio® is indicated for the treatment of severe hepatic Veno-Occlusive Disease (sVOD) also known as sinusoidal obstruction syndrome (SOS) in haematopoietic stem-cell transplantation (HSCT) therapy.

It is indicated in adults and in adolescents, children and infants over 1 month of age.

As required by the risk management plan, Gentium set up a disease registry to collect safety and outcome data, and to assess patterns of utilization of Defitelio® in the post-approval setting.

This is a multi-centre, multinational and prospective observational (non-interventional) disease registry of patients with severe hepatic VOD following HSCT and treated with Defitelio®.

The registry will be conducted in European transplant centres that are members of the European Society for Blood and Marrow Transplantation (EBMT).

The main objective of the registry is to assess the incidence rate of specific SAEs (Serious Adverse Events) of interest (including fatalities) in patients with severe hepatic VOD post-HSCT treated with Defitelio®.

Secondary objectives are:

* To describe the population treated with Defibrotide (age, gender, patients with pre-existing liver or severe renal insufficiency; patient with intrinsic lung disease)

* To determine the incidence rate of multiorgan failure (MOF) and Graft versus host disease (GvHD) in adult and paediatric patients receiving Defibrotide.

* To determine survival by Day+100 post-HSCT, overall mortality and mortality due to VOD in patients treated with Defibrotide.

* To determine the rate of VOD and VOD with MOF resolution any time after treatment initiation in patients treated with Defibrotide.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
176
Inclusion Criteria
  • Patients undergoing hematopoietic stem cell transplantation and diagnosed with severe hepatic VOD, who agree to participate in the study (Main population).
  • Patients treated with defibrotide for another condition than severe hepatic VOD (Secondary population) in the scope of hematopoietic stem cell transplantation .
Exclusion Criteria
  • There will not be any specific exclusion criteria; however contraindications, special warnings and precautions for use as detailed in the SPC will have to be considered by the treating physician.

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Incidence rate of specific SAE of interestover 12 months

Assess the incidence rate of specific SAEs of interest (including fatalities) in patients with severe hepatic VOD post-HSCT treated with Defitelio®

Secondary Outcome Measures
NameTimeMethod
Survivalover 12 months

To determine survival by Day+100 post-HSCT, overall mortality and mortality due to VOD in patients treated with Defitelio®

GvHD incidenceover 12 months

Determine the incidence rate of Graft versus host disease (GvHD) in adult and paediatric patients receiving Defitelio®

Describe the population treatedEnrolment

To describe the population treated with Defitelio® (age, gender, patients with pre-existing liver or severe renal insufficiency; patient with intrinsic lung disease)

VOD/MOF Resolutionover 12 months

To determine the rate of VOD and VOD with MOF resolution any time after treatment initiation in patients treated with Defitelio®

Trial Locations

Locations (53)

CHU d'Estaing

🇫🇷

Clermont Ferrand, France

IHOP

🇫🇷

Lyon, France

Hôpital d'enfants de la Timone

🇫🇷

Mare aux Daims, France

Institut Paoli Calmette

🇫🇷

Marseille, France

CHU Lapeyronie

🇫🇷

Montpellier, France

Hôpital de l'ARCHET

🇫🇷

Nice, France

Hôpital Saint Antoine

🇫🇷

Paris, France

Hôpital Robert Debré

🇫🇷

Paris, France

Hôpital Tenon

🇫🇷

Paris, France

Institut Curie

🇫🇷

Paris, France

Hôpital Haut Leveque

🇫🇷

Pessac, France

Hôpital de la Miletrie

🇫🇷

Poitiers, France

CHU Hautepierre

🇫🇷

Strasbourg, France

IUTC Oncopole

🇫🇷

Toulouse, France

Institute G. Gaslini

🇮🇹

Genova, Italy

Ospedale Ca' Granda Ospedale Maggiore di Milano

🇮🇹

Milano, Italy

Ospedale San Raffaele

🇮🇹

Milan, Italy

Universita Cattolica S. Cuore

🇮🇹

Roma, Italy

IRRCS Ospedale Pediatrico Bambino Gesù

🇮🇹

Roma, Italy

AOU Citta della salute e delle Scienza

🇮🇹

Torino, Italy

Ospedale Infantile Regina Margherita

🇮🇹

Torino, Italy

Istituto per l'Infanzia IRCCS Burlo Garofolo

🇮🇹

Trieste, Italy

Clinica Ematologica-Azienda Ospedaliero Universitaria

🇮🇹

Udine, Italy

Birmingham Children's Hospital

🇬🇧

Birmingham, United Kingdom

Birmingham Heartlands Hospital

🇬🇧

Birmingham, United Kingdom

Bristol Royal Hospital for Children

🇬🇧

Bristol, United Kingdom

Great Ormond Street Hospital Children's Charity

🇬🇧

London, United Kingdom

Inst. Portugues Oncologia o Lisboa

🇵🇹

Lisboa, Portugal

CHU Bordeaux Groupe hospitalier Pellegrin Enfants

🇫🇷

Bordeaux, France

CHRU Angers, Maladies du Sang

🇫🇷

Angers, France

CHRU Limoges

🇫🇷

Limoges, France

CHRU de Lille

🇫🇷

Lille, France

Hôpital Jeanne de Flandre

🇫🇷

Lille, France

CHU Nantes

🇫🇷

Nantes, France

Institut Gustave Roussy (Pediatrics)

🇫🇷

Villejuif, France

Azienda Ospedaliera Papa Giovanni XXIII

🇮🇹

Bergamo, Italy

Institut Gustave Roussy

🇫🇷

Villejuif, France

UO Oncoematologia Ospedale Pediatrico Mayer

🇮🇹

Firenze, Italy

Ospedale di Careggi

🇮🇹

Florence, Italy

Ospedale di Niguarda Ca' Granda

🇮🇹

Milano, Italy

Ospedale Santa Maria de la Misericorda

🇮🇹

Perugia, Italy

Ospedale San Carlo

🇮🇹

Potenza, Italy

Clinica di oncoematologia pediatrica

🇮🇹

Padova, Italy

La Sapienza University Hospital

🇮🇹

Rome, Italy

Policlinico G.B. Rossi, Clinica di Oncoematologia Pediatrica

🇮🇹

Verona, Italy

Fondazione IRCCS Policlinico San Matteo

🇮🇹

Pavia, Italy

Royal Manchester Children's Hospital

🇬🇧

Manchester, United Kingdom

Great North Children's Hospital

🇬🇧

Newcastle Upon Tyne, United Kingdom

Imperial College

🇬🇧

London, United Kingdom

John Radccliffe Children's Hospital

🇬🇧

Oxford, United Kingdom

Nottingham City Hospital

🇬🇧

Nottingham, United Kingdom

Plymouth Hospitals NHS Trust

🇬🇧

Plymouth, United Kingdom

St George's Hospital

🇬🇧

London, United Kingdom

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