Survey of Inhibitors in Plasma-Product Exposed Toddlers

NCT ID: NCT01064284

Last Updated: 2017-08-25

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE4

Total Enrollment

303 participants

Study Classification

INTERVENTIONAL

Study Start Date

2010-01-31

Study Completion Date

2015-05-31

Brief Summary

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The primary objective of the study is to assess the immunogenicity of VWF/FVIII and of rFVIII concentrates by determining the frequency of inhibitor development in previously untreated patients (PUPs) or minimally blood component-treated (MBCTPs) in the first 50 EDs or in the first 3 years from enrollment, whichever occurs first.

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Detailed Description

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Patients meeting the enrollment criteria will be consecutively enrolled at each participating centre, randomized to be treated exclusively with a single FVIII product either plasma-derived or recombinant, and followed up until inhibitor development or until 50 exposure days (EDs) or 3 years from enrolment have elapsed, whichever comes first. Study products, belonging to the class of rFVIII concentrates and to the class of plasma-derived VWF/FVIII concentrates, will be provided for free to the patients for all the duration of the study

Conditions

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Hemophilia A

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

BASIC_SCIENCE

Blinding Strategy

NONE

Study Groups

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PLASMA DERIVED Factor VIII

Plasma-derived vWF/FVIII

Group Type ACTIVE_COMPARATOR

PLASMA DERIVED Factor VIII

Intervention Type DRUG

Maximum dosage : 50IU per kilo. 2-3 times per week or on demand during acute episode of bleeding

rFVIII

Recombinant FVIII

Group Type ACTIVE_COMPARATOR

Recombinant FVIII

Intervention Type DRUG

Maximum dosage : 50IU per kilo. 2-3 times per week or on demand during acute episode of bleeding

Interventions

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PLASMA DERIVED Factor VIII

Maximum dosage : 50IU per kilo. 2-3 times per week or on demand during acute episode of bleeding

Intervention Type DRUG

Recombinant FVIII

Maximum dosage : 50IU per kilo. 2-3 times per week or on demand during acute episode of bleeding

Intervention Type DRUG

Other Intervention Names

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ALPHANATE ADVATE

Eligibility Criteria

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Inclusion Criteria

* Male subjects
* Any ethnicity
* Age \<6 years
* Severe haemophilia A (FVIII:C \<1%), as confirmed at enrolment by the central laboratory.

o Those patients diagnosed locally as severe but subsequently found to have FVIII levels \>= 1% on testing at the central laboratory will be separately recorded in the screening list.
* Previously untreated (0 EDs to any FVIII concentrates or blood products) or minimally treated (\<5 EDs) with blood components, namely whole blood, fresh frozen plasma, packed red blood cells, platelets or cryoprecipitate.

o Patients not meeting these criteria will be separately recorded in the screening list.
* Negative inhibitor measurement at both local and central laboratory at screening
* Ability to comply with study requirements
* Signed informed consent of legal tutors o Patients who will not accept to enter into the study or to be randomized will be separately recorded.

Exclusion Criteria

* Previous history of FVIII inhibitor
* Other congenital or acquired bleeding defects
* Plasma FVIII level \>= 1%, as assayed at the central laboratory

o Those patients originally diagnosed locally as severe but subsequently found to have FVIII levels ranging from 1% to 2% on testing at the central laboratory will be separately recorded in the screening list.
* Concomitant congenital or acquired immunodeficiency
* Concomitant treatment with systemic immunosuppressive drugs
* Concomitant treatment with any investigational drug
Minimum Eligible Age

1 Minute

Maximum Eligible Age

6 Years

Eligible Sex

MALE

Accepts Healthy Volunteers

No

Sponsors

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Sintesi Research Srl

INDUSTRY

Sponsor Role collaborator

Fondazione Angelo Bianchi Bonomi

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Pier M. Mannucci, Professor

Role: PRINCIPAL_INVESTIGATOR

Fondazione Ca' Granda Ospedale Maggiore Policlinico Milano

Flora Peyvandi, Professor

Role: PRINCIPAL_INVESTIGATOR

Fondazione Ca' Granda Ospedale Maggiore Policlinico Milano

Locations

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City of Hope National Medical Center

Duarte, California, United States

Site Status

Children's Hospital Los Angeles (CHLA)

Los Angeles, California, United States

Site Status

Hemophilia and Thrombosis CenterUniversity of Colorado Denver - Anschutz Aurora

Aurora, Colorado, United States

Site Status

Rush Hemophilia & Trombophilia Center - Rush University Medical Center

Chicago, Illinois, United States

Site Status

Louisiana Center for Bleeding and Clotting Disorders, Tulane University Medical Center

New Orleans, Louisiana, United States

Site Status

University of Mississippi Medical Center, Division of pediatric Hematology/Oncology

Jackson, Mississippi, United States

Site Status

Children's Mercy Hospital

Kansas City, Missouri, United States

Site Status

Hemophilia Treatment Center of Las Vegas

Las Vegas, Nevada, United States

Site Status

MeritCare Roger Maris Cancer Center, Pediatric Oncology

Fargo, North Dakota, United States

Site Status

Cook Children's Medical Center

Fort Worth, Texas, United States

Site Status

Hospital de Ninos Sor Maria Ludovica La Plata Servicio de Hematologia

La Plata, Buenos Aires, Argentina

Site Status

Fundacion de la Hemofilia

Buenos Aires, , Argentina

Site Status

Landes- Frauen- und Kinderklinik Linz Abteilung für Kinder- und Jugendheilkunde

Linz, , Austria

Site Status

Medizinische Universität Wien, Dept. Paediatrics

Vienna, , Austria

Site Status

Centro de Pesquisa Clinica HEMORIO - Instituto Estadual de Hematologia Arthur de Siqueira Cavalcanti

Rio de Janeiro, , Brazil

Site Status

Centro de Hematologia e Hemoterapia do e.s - Hemoes

Vitória, , Brazil

Site Status

Hospital de Niños Dr. Luis Calvo Mackenna Centro Hemofílico

Santiago, , Chile

Site Status

Centro de Hemofilicos del Hospital de Niños Roberto del Rio Instituto de Investigaciones Hematologicas

Santiago, , Chile

Site Status

Paediatric Haematology department, Cairo University Pediatric Hospital

Cairo, , Egypt

Site Status

Faculty of Medicine Ain Shams University Department Pediatrics

Cairo, , Egypt

Site Status

Centre for Blood Disorders

Chennai, , India

Site Status

St. John's Medical College & Hospital

Karnataka, , India

Site Status

Kasturba Medical College, Manipal University

Karnataka, , India

Site Status

Karnataka Hemophilia Care and Hematology Research Center

Karnataka, , India

Site Status

Kerala Institute of Medical Science (KIMS)

Kerala, , India

Site Status

Lokmanya TilakMunicipal Medical College &General Hospital - Sion

Mumbai, , India

Site Status

All India Institute of Medical Sciences Department of Haematology

New Delhi, , India

Site Status

Sir Ganga Ram Hospital

New Delhi, , India

Site Status

Sahyadri Speciality Hospital

Pune, , India

Site Status

Jehangir Clinical Development Centre, Department of Haematology, Jehangir Hospital Premises

Pune, , India

Site Status

Hemophilia Center - Hematoogy & Oncology Dept. Shiraz University of Medical Science Ayatollah Dastgheib Hospital

Shiraz, , Iran

Site Status

Comprehensive Care Center for Children with Hemophilia Mofid Children Hospital

Tehran, , Iran

Site Status

Centro Emofilia e Trombosi "Angelo Bianchi Bonomi" Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano - Italy

Milan, , Italy

Site Status

Clinica Medica II - Azienda Ospedaliera di Padova - Centro Emofilia di Padova

Padua, , Italy

Site Status

Ematologia- UO Diagnostica Speciale e Terapia delle Malattie dell'Emostasi e della Trombosi- Università Sapienza - Policlinico Umberto I

Rome, , Italy

Site Status

Unidad Medica de Alta Especialidad (UMAE), Hospital de Pediatria. Centro Medico Nacional de Occidente Istituto Mexicano del Seguro Social

Jalisco, , Mexico

Site Status

Instituto Nacional de Pediatria

México D.F., , Mexico

Site Status

Hospital Infantil de Mexico Federico Gomez

México, D.F., , Mexico

Site Status

Hospital Universitario Dr. Josè Eleuterio Gonzalez de la UANL, NL. Mexico

Monterrey, , Mexico

Site Status

Hospital de Especialidades UMAE Istituto Mexicano del Seguro Social (IMSS)

Monterrey (Nuevo Leòn), , Mexico

Site Status

Kinf Faisal Specilist Hospital and Research Center

Riyadh, , Saudi Arabia

Site Status

Haemophilia Comprehensive Care Clinic, Area 454, Charlotte Maxeke Johannesburg Academic Hospital

Parktown, , South Africa

Site Status

Hospital Regional Universitario Carlos Haya

Málaga, , Spain

Site Status

Hospital Universitario Virgen del Rocio Unidad de Hemofilia

Seville, , Spain

Site Status

Hospital Universitario La Fe Unidad Coagulopatias Congenitas

Valencia, , Spain

Site Status

Cukurova Universitesi, Tip Fakultesi Pediatrik Hematoloji B.D.

Adana, , Turkey (Türkiye)

Site Status

Ege Üniversitesi Tip Fakültesi Cocuk Sağliği ve Hastalikari Anabilim Dali Pediatrik Hematoloji Bilim Dali

Bornova/Izmir, , Turkey (Türkiye)

Site Status

Istanbul Üniversitesi Cerrahpaşa Tip Fakültesi Pediatrik Hematoloji B.D.

Istanbul, , Turkey (Türkiye)

Site Status

Countries

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United States Argentina Austria Brazil Chile Egypt India Iran Italy Mexico Saudi Arabia South Africa Spain Turkey (Türkiye)

References

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Addiego J, Kasper C, Abildgaard C, Hilgartner M, Lusher J, Glader B, Aledort L. Frequency of inhibitor development in haemophiliacs treated with low-purity factor VIII. Lancet. 1993 Aug 21;342(8869):462-4. doi: 10.1016/0140-6736(93)91593-b.

Reference Type BACKGROUND
PMID: 8102429 (View on PubMed)

Amano K, Arai M, Koshihara K, Suzuki T, Kagawa K, Nishida Y, Fukutake K. Autoantibody to factor VIII that has less reactivity to factor VIII/von Willebrand factor complex. Am J Hematol. 1995 Aug;49(4):310-7. doi: 10.1002/ajh.2830490409.

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Auerswald G, Spranger T, Brackmann HH. The role of plasma-derived factor VIII/von Willebrand factor concentrates in the treatment of hemophilia A patients. Haematologica. 2003 Jun;88(6):EREP05.

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Chalmers EA, Brown SA, Keeling D, Liesner R, Richards M, Stirling D, Thomas A, Vidler V, Williams MD, Young D; Paediatric Working Party of UKHCDO. Early factor VIII exposure and subsequent inhibitor development in children with severe haemophilia A. Haemophilia. 2007 Mar;13(2):149-55. doi: 10.1111/j.1365-2516.2006.01418.x.

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Courter SG, Bedrosian CL. Clinical evaluation of B-domain deleted recombinant factor VIII in previously untreated patients. Semin Hematol. 2001 Apr;38(2 Suppl 4):52-9. doi: 10.1016/s0037-1963(01)90109-x.

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Dasgupta S, Repesse Y, Bayry J, Navarrete AM, Wootla B, Delignat S, Irinopoulou T, Kamate C, Saint-Remy JM, Jacquemin M, Lenting PJ, Borel-Derlon A, Kaveri SV, Lacroix-Desmazes S. VWF protects FVIII from endocytosis by dendritic cells and subsequent presentation to immune effectors. Blood. 2007 Jan 15;109(2):610-2. doi: 10.1182/blood-2006-05-022756. Epub 2006 Sep 19.

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Goudemand J, Rothschild C, Demiguel V, Vinciguerrat C, Lambert T, Chambost H, Borel-Derlon A, Claeyssens S, Laurian Y, Calvez T; FVIII-LFB and Recombinant FVIII study groups. Influence of the type of factor VIII concentrate on the incidence of factor VIII inhibitors in previously untreated patients with severe hemophilia A. Blood. 2006 Jan 1;107(1):46-51. doi: 10.1182/blood-2005-04-1371. Epub 2005 Sep 15.

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Gouw SC, van der Bom JG, Auerswald G, Ettinghausen CE, Tedgard U, van den Berg HM. Recombinant versus plasma-derived factor VIII products and the development of inhibitors in previously untreated patients with severe hemophilia A: the CANAL cohort study. Blood. 2007 Jun 1;109(11):4693-7. doi: 10.1182/blood-2006-11-056317. Epub 2007 Jan 11.

Reference Type BACKGROUND
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Gringeri A, Mantovani LG, Scalone L, Mannucci PM; COCIS Study Group. Cost of care and quality of life for patients with hemophilia complicated by inhibitors: the COCIS Study Group. Blood. 2003 Oct 1;102(7):2358-63. doi: 10.1182/blood-2003-03-0941. Epub 2003 Jun 19.

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Gringeri A, Monzini M, Tagariello G, Scaraggi FA, Mannucci PM; Emoclot15 Study Members. Occurrence of inhibitors in previously untreated or minimally treated patients with haemophilia A after exposure to a plasma-derived solvent-detergent factor VIII concentrate. Haemophilia. 2006 Mar;12(2):128-32. doi: 10.1111/j.1365-2516.2006.01201.x.

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Guerois C, Laurian Y, Rothschild C, Parquet-Gernez A, Duclos AM, Negrier C, Vicariot M, Fimbel B, Fressinaud E, Fiks-Sigaud M, et al. Incidence of factor VIII inhibitor development in severe hemophilia A patients treated only with one brand of highly purified plasma-derived concentrate. Thromb Haemost. 1995 Feb;73(2):215-8.

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Kreuz W, Gill JC, Rothschild C, Manco-Johnson MJ, Lusher JM, Kellermann E, Gorina E, Larson PJ; International Kogenate-FS Study Group. Full-length sucrose-formulated recombinant factor VIII for treatment of previously untreated or minimally treated young children with severe haemophilia A: results of an international clinical investigation. Thromb Haemost. 2005 Mar;93(3):457-67. doi: 10.1160/TH03-10-0643.

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Lorenzo JI, Lopez A, Altisent C, Aznar JA. Incidence of factor VIII inhibitors in severe haemophilia: the importance of patient age. Br J Haematol. 2001 Jun;113(3):600-3. doi: 10.1046/j.1365-2141.2001.02828.x.

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Lusher JM, Arkin S, Abildgaard CF, Schwartz RS. Recombinant factor VIII for the treatment of previously untreated patients with hemophilia A. Safety, efficacy, and development of inhibitors. Kogenate Previously Untreated Patient Study Group. N Engl J Med. 1993 Feb 18;328(7):453-9. doi: 10.1056/NEJM199302183280701.

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Qadura M, Waters B, Burnett E, Chegeni R, Othman M, Lillicrap D. Investigating the mechanisms underlying FVIII antibody production in hemophilic mice following recombinant and plasma-derived FVIII infusion. Blood (ASH Annual Meeting Abstracts) 2008; 112: abstract #237.

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Rothschild C, Laurian Y, Satre EP, Borel Derlon A, Chambost H, Moreau P, Goudemand J, Parquet A, Peynet J, Vicariot M, Beurrier P, Claeyssens S, Durin A, Faradji A, Fressinaud E, Gaillard S, Guerin V, Guerois C, Pernod G, Pouzol P, Schved JF, Gazengel C. French previously untreated patients with severe hemophilia A after exposure to recombinant factor VIII : incidence of inhibitor and evaluation of immune tolerance. Thromb Haemost. 1998 Nov;80(5):779-83.

Reference Type BACKGROUND
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Scharrer I, Ehrlich HJ. Reported inhibitor incidence in FVIII PUP studies: comparing apples with oranges? Haemophilia. 2004 Mar;10(2):197-8. doi: 10.1111/j.1365-2516.2004.00887.x. No abstract available.

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Strauss T, Lubetsky A, Ravid B, Bashari D, Luboshitz J, Lalezari S, Misgav M, Martinowitz U, Kenet G. Recombinant factor concentrates may increase inhibitor development: a single centre cohort study. Haemophilia. 2011 Jul;17(4):625-9. doi: 10.1111/j.1365-2516.2010.02464.x. Epub 2011 Feb 7.

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Waters B, Qadura M, Burnett E, Chegeni R, Labelle A, Thompson P, Hough C, Lillicrap D. Anti-CD3 prevents factor VIII inhibitor development in hemophilia A mice by a regulatory CD4+CD25+-dependent mechanism and by shifting cytokine production to favor a Th1 response. Blood. 2009 Jan 1;113(1):193-203. doi: 10.1182/blood-2008-04-151597. Epub 2008 Sep 24.

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Wight J, Paisley S. The epidemiology of inhibitors in haemophilia A: a systematic review. Haemophilia. 2003 Jul;9(4):418-35. doi: 10.1046/j.1365-2516.2003.00780.x.

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PMID: 12828678 (View on PubMed)

Rosendaal FR, Palla R, Garagiola I, Mannucci PM, Peyvandi F; SIPPET Study Group. Genetic risk stratification to reduce inhibitor development in the early treatment of hemophilia A: a SIPPET analysis. Blood. 2017 Oct 12;130(15):1757-1759. doi: 10.1182/blood-2017-06-791756. Epub 2017 Aug 2.

Reference Type DERIVED
PMID: 28768627 (View on PubMed)

Peyvandi F, Mannucci PM, Garagiola I, El-Beshlawy A, Elalfy M, Ramanan V, Eshghi P, Hanagavadi S, Varadarajan R, Karimi M, Manglani MV, Ross C, Young G, Seth T, Apte S, Nayak DM, Santagostino E, Mancuso ME, Sandoval Gonzalez AC, Mahlangu JN, Bonanad Boix S, Cerqueira M, Ewing NP, Male C, Owaidah T, Soto Arellano V, Kobrinsky NL, Majumdar S, Perez Garrido R, Sachdeva A, Simpson M, Thomas M, Zanon E, Antmen B, Kavakli K, Manco-Johnson MJ, Martinez M, Marzouka E, Mazzucconi MG, Neme D, Palomo Bravo A, Paredes Aguilera R, Prezotti A, Schmitt K, Wicklund BM, Zulfikar B, Rosendaal FR. A Randomized Trial of Factor VIII and Neutralizing Antibodies in Hemophilia A. N Engl J Med. 2016 May 26;374(21):2054-64. doi: 10.1056/NEJMoa1516437.

Reference Type DERIVED
PMID: 27223147 (View on PubMed)

Study Documents

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Document Type: Article

View Document

Related Links

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http://sippetstudy.org/

The official web site of the SIPPET Study

Other Identifiers

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2009-011186-88

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

ABB - 09 - 001

Identifier Type: -

Identifier Source: org_study_id

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