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1. Consensus statements on vaccination in patients with haemophilia—Results from the Italian haemophilia and vaccinations (HEVA) project

Author

Santagostino E., Riva A., Cesaro S., Esposito S., Matino D., Mazzucchelli R. I., Molinari, Angelo Claudio, Mura R., Notarangelo L. D., Tagliaferri A., Di Minno G., Clerici M., Ambaglio C., Brigida Aru A., Baldacci E., Barillari G., Basso M., Bernasconi S., Bertamino M., Bertoni E., Biasoli C., Federica Biguzzi E., Bonetti E., Borchiellini A., Bulgarelli S., Cabibbo S., Cantori I., Castaman G., Castiglia P., Coluccia A., Coppetelli U., Coppola A., Cultrera D., De Candia E., Delios G., Di Gennaro L., Di Gregorio P., Di Minno M., Dragani A., Pietro Ettorre C., Franchini M., Galli M., Gallo G., Giordano P., Giuffrida G., Iannaccaro P., Lassandro G., Lazzareschi I., Linari S., Luciani M., Macchi S., Malcangi G., Malizia R., Marietta M., Marino R., Massoud M., Gabriella Mazzucconi M., Milan M., Morfini M., Napolitano M., Pasca S., Pedrazzi P., Peyvandi F. A., Piscitelli L., Pollio B., Preti P., Quintavalle G., Radossi P., Raso S., Ricca I., Rocino A., Santoro C., Carlotta Santoro R., Sarolo L., Schiavoni M., Schiavulli M., Sciancalepore P., Luisa Serino M., Mario Siragusa S., Sottilotta G., Svahn J., Valdre L., Cristina Vedovati M., Zanon E., Santagostino, E., Riva, A., Cesaro, S., Esposito, S., Matino, D., Mazzucchelli, R. I., Molinari, Angelo Claudio, Mura, R., Notarangelo, L. D., Tagliaferri, A., Di Minno, G., Clerici, M., Ambaglio, C., Brigida Aru, A., Baldacci, E., Barillari, G., Basso, M., Bernasconi, S., Bertamino, M., Bertoni, E., Biasoli, C., Federica Biguzzi, E., Bonetti, E., Borchiellini, A., Bulgarelli, S., Cabibbo, S., Cantori, I., Castaman, G., Castiglia, P., Coluccia, A., Coppetelli, U., Coppola, A., Cultrera, D., De Candia, E., Delios, G., Di Gennaro, L., Di Gregorio, P., Di Minno, M., Dragani, A., Pietro Ettorre, C., Franchini, M., Galli, M., Gallo, G., Giordano, P., Giuffrida, G., Iannaccaro, P., Lassandro, G., Lazzareschi, I., Linari, S., Luciani, M., Macchi, S., Malcangi, G., Malizia, R., Marietta, M., Marino, R., Massoud, M., Gabriella Mazzucconi, M., Milan, M., Morfini, M., Napolitano, M., Pasca, S., Pedrazzi, P., Peyvandi, F. A., Piscitelli, L., Pollio, B., Preti, P., Quintavalle, G., Radossi, P., Raso, S., Ricca, I., Rocino, A., Santoro, C., Carlotta Santoro, R., Sarolo, L., Schiavoni, M., Schiavulli, M., Sciancalepore, P., Luisa Serino, M., Mario Siragusa, S., Sottilotta, G., Svahn, J., Valdre, L., Cristina Vedovati, M., and Zanon, E.

Subjects
Adult, medicine.medical_specialty, Consensus, Delphi Technique, Vaccination schedule, Delphi method, haemophilia, 030204 cardiovascular system & hematology, Hemophilia A, Haemophilia, immunization, Hemophilia B, bleeding disorder, factor VIII inhibitor, vaccination, Child, Evidence-Based Medicine, Humans, Italy, Vaccination, 03 medical and health sciences, 0302 clinical medicine, Vaccine administration, Medicine, In patient, Clinical Haemophilia, Genetics (clinical), business.industry, Original Articles, Hematology, General Medicine, Evidence-based medicine, medicine.disease, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Immunization, Family medicine, Original Article, business, 030215 immunology
Abstract

Vaccination against communicable diseases is crucial for disease prevention, but this practice poses challenges to healthcare professionals in patients with haemophilia. Poor knowledge of the vaccination requirements for these patients and safety concerns often result in vaccination delay or avoidance. In order to address this issue, a panel of 11 Italian haemophilia and immunization experts conducted a Delphi consensus process to identify the main concerns regarding the safe use of vaccines in patients with haemophilia. The consensus was based on a literature search of the available evidence, which was used by the experts to design 27 consensus statements. A group of clinicians then rated these statements using the 5‐point Likert‐type scale (1 = strongly disagree; 5 = strongly agree). The main issues identified by the expert panel included vaccination schedule for haemophilic patients; protocol and optimal route of vaccine administration; vaccination of haemophilic patients with antibodies inhibiting coagulation factor VIII (inhibitors); and vaccination and risk of inhibitor development. This manuscript discusses these controversial areas in detail supported by the available literature evidence and provides evidence‐ and consensus‐based recommendations. Overall, participants agreed on most statements, except those addressing the potential role of vaccination in inhibitor formation. Participants agreed that patients with haemophilia should receive vaccinations according to the institutional schedule for individuals without bleeding disorders; however, vaccination of patients with haemophilia requires comprehensive planning, taking into account disease severity, type and route of vaccination, and bleeding risk. Data also suggest vaccination timing does not need to take into consideration when the patient received factor VIII replacement.

Published
2019

2. Perceived challenges and attitudes to regimen and product selection from Italian haemophilia treaters: the 2013 AICE survey

Author

Franchini, M, Coppola, A, Rocino, A, Zanon, E, Morfini, M, Italian Association of Haemophilia Centers AICE Working Group, Accorsi, A, Aru, Ab, Biasoli, C, Cantori, I, Castaman, G, Cesaro, S, Ciabatta, C, De Cristofaro, R, Delios, G, Di Minno, G, D'Incà, M, Dragani, A, Ettorre, Cp, Gagliano, F, Gamba, G, Gandini, G, Giordano, P, Giuffrida, G, Gresele, P, Latella, C, Luciani, M, Margaglione, M, Marietta, M, Mazzucconi, Maria Gabriella, Messina, M, Molinari, Ac, Notarangelo, Ld, Oliovecchio, E, Peyvandi, F, Piseddu, G, Rossetti, G, Rossi, V, Santagostino, E, Schiavoni, M, Schinco, P, Serino, Ml, Tagliaferri, A, Testa, S., Franchini, M, Coppola, A, Rocino, A, Zanon, E, Morfini, M, Accorsi, A, Aru, Ab, Biasoli, C, Cantori, I, Castaman, G, Cesaro, S, Ciabatta, C, De Cristofaro, R, Delios, G, DI MINNO, Giovanni, D'Incà, M, Dragani, A, Ettorre, Cp, Gagliano, F, Gamba, G, Gandini, G, Giordano, P, Giuffrida, G, Gresele, P, Latella, C, Luciani, M, Margaglione, M, Marietta, M, Mazzucconi, Mg, Messina, M, Molinari, Ac, Notarangelo, Ld, Oliovecchio, E, Peyvandi, F, Piseddu, G, Rossetti, G, Rossi, V, Santagostino, E, Schiavoni, M, Schinco, P, Serino, Ml, Tagliaferri, A, and Testa, S.

Subjects
Haemophilia, Pediatrics, medicine.medical_specialty, factor concentrates, haemophilia treatment, inhibitor, prophylaxis, survey, viral safety, Blood Coagulation Factors, Health Care Surveys, Hemophilia A, Humans, Italy, Practice Patterns, Physicians', Surveys and Questionnaires, Hospitals, Special, Medical Staff, Hospital, Practice Patterns, NO, Hospital, Haemophilia A, Medical Staff, Medicine, Genetics (clinical), Clotting factor, Physicians', Special, Treatment regimen, business.industry, Settore MED/09 - MEDICINA INTERNA, Hematology, General Medicine, medicine.disease, Blood coagulation factors, Hospitals, Clinical Practice, Regimen, Family medicine, Product selection, business
Abstract

Summary Despite great advances in haemophilia care in the last 20 years, a number of questions on haemophilia therapy remain unanswered. These debated issues primarily involve the choice of the product type (plasma-derived vs. recombinant) for patients with different characteristics: specifically, if they were infected by blood-borne virus infections, and if they bear high or low risk of inhibitor development. In addition, the most appropriate treatment regimen in non-inhibitor and inhibitor patients compel physicians operating at the haemophilia treatment centres (HTCs) to take important therapeutic decisions, which are often based on their personal clinical experience rather than on evidence-based recommendations from published literature data. To know the opinion on the most controversial aspects in haemophilia care of Italian expert physicians, who are responsible for common clinical practice and therapeutic decisions, we have conducted a survey among the Directors of HTCs affiliated to the Italian Association of Haemophilia Centres (AICE). A questionnaire, consisting of 19 questions covering the most important topics related to haemophilia treatment, was sent to the Directors of all 52 Italian HTCs. Forty Directors out of 52 (76.9%) responded, accounting for the large majority of HTCs affiliated to the AICE throughout Italy. The results of this survey provide for the first time a picture of the attitudes towards clotting factor concentrate use and product selection of clinicians working at Italian HTCs.

Published
2014

3. Immune tolerance induction with a high purity von Willebrand factor/VIII complex concentrate in haemophilia A patients with inhibitors at high risk of a poor response

Author

Gringeri, A., Musso, R., Mazzucconi, Maria Gabriella, Piseddu, G., Schiavoni, M., Pignoloni, P., Mannucci, P. M., and For The Rits Fitnhes Study Group

Subjects
Adult, medicine.medical_specialty, Adolescent, Haemophilia A, Hemophilia A, Haemophilia, Risk Assessment, Gastroenterology, haemophilia a, immune tolerance, inhibitors, rescue treatment, vwf, Immune tolerance, Von Willebrand factor, Internal medicine, von Willebrand Factor, Immune Tolerance, medicine, Humans, In patient, Child, Genetics (clinical), Immunosuppression Therapy, Blood Coagulation Factor Inhibitors, Dose-Response Relationship, Drug, biology, business.industry, Hematology, General Medicine, Middle Aged, medicine.disease, Surgery, Titer, Dose–response relationship, Child, Preschool, biology.protein, Bypassing agent, business
Abstract

Immune tolerance induction (ITI) is effective in approximately 70% of haemophilia patients with inhibitors. Poor prognostic factors are age >6 years, ITI started >1 year from inhibitor development, inhibitor peaks >200 BU, inhibitor titre >10 BU when ITI is started and previously failed ITI. The objective of this study was to identify the effectiveness in ITI of a high purity von Willebrand factor/factor VIII (VWF/FVIII) complex concentrate in inhibitor patients at high risk of failure. Patients with severe or moderate haemophilia A and high responding inhibitors who had at least one poor prognostic factor for ITI failure were prospectively followed-up. Success was defined by undetectable inhibitor, recovery and half life >66% of expected values. ITI dose regimens were chosen by each haemophilia centre. Seventeen haemophiliacs (16 severe, one moderate), aged 4-54 years (median 23) were followed-up for 6-71 months. Poor prognostic factors were delayed-onset ITI (n = 16), age >6 years (n = 16), previously failed ITI (n = 4), inhibitor peak >200 BU (n = 2) and inhibitor >10 BU when ITI was started (n = 4). Complete success was obtained in nine patients (53%) after 4-30 months of treatment (median 24), including two of four patients who had previously failed ITI. Seven patients achieved a partial success, with sustained low inhibitor titres (median 1.5 BU, range 1.1-2.8) but abnormal recovery and/or half-life, while the remaining patient withdrew ITI after 12 months when the inhibitor titer was still 70 BU. These findings suggest that high purity VWF/FVIII complex concentrates are effective in ITI, even in patients at high risk of failure.

Published
2007

4. Consumption of clotting factors in severe haemophilia patients undergoing prophylaxis and on-demand treatment in Italy

Author

Giampaolo A, Abbonizio F, Puopolo M, Arcieri R, Mannucci PM, Hassan HJ, Accorsi A, Ettorre PC, Schiavoni M, Palareti G, Rodorigo G, Valdré L, Amaddii G, Billio A, Notarangelo L, Iannacaro P, Muleo P, Biasioli C, Rossi V, Alatri A, Testa S, Vincenzi D, Scapoli G, Morfini M, Molinari AC, Lapecorella M, Mariani G, Baudo F, Caimi MT, Federici AB, Gringeri A, Santagostino E, Marietta M, Coppola A, Perricone C, Schiavulli M, Miraglia E, Rocino A, Zanon E, Gagliano F, Mancuso G, Siragusa S, Rivolta F, Tagliaferri A, Gamba G, Iorio A, Oliovecchio E, Dragani A, Arbasi MC, Albertini P, Mancino A, Lombardo VT, Latella C, D'Incà M, Landolfi Raffaele, Biondo Francesca, Mazzucconi MG, Santoro Cristina, Mameli AL, Piseddu G, Schinco PC, Messina M, Rossetti G, Barillari G, Feola G, Franchini M, Gandini G, Castaman G, Rodeghiero F, Italian Association of Haemophilia Centres, DI MINNO, GIOVANNI, Giampaolo, A, Abbonizio, F, Puopolo, M, Arcieri, R, Mannucci, Pm, Hassan, Hj, Accorsi, A, Ettorre, Pc, Schiavoni, M, Palareti, G, Rodorigo, G, Valdré, L, Amaddii, G, Billio, A, Notarangelo, L, Iannacaro, P, Muleo, P, Biasioli, C, Rossi, V, Alatri, A, Testa, S, Vincenzi, D, Scapoli, G, Morfini, M, Molinari, Ac, Lapecorella, M, Mariani, G, Baudo, F, Caimi, Mt, Federici, Ab, Gringeri, A, Santagostino, E, Marietta, M, Coppola, A, DI MINNO, Giovanni, Perricone, C, Schiavulli, M, Miraglia, E, Rocino, A, Zanon, E, Gagliano, F, Mancuso, G, Siragusa, S, Rivolta, F, Tagliaferri, A, Gamba, G, Iorio, A, Oliovecchio, E, Dragani, A, Arbasi, Mc, Albertini, P, Mancino, A, Lombardo, Vt, Latella, C, D'Incà, M, Landolfi, Raffaele, Biondo, Francesca, Mazzucconi, Mg, Santoro, Cristina, Mameli, Al, Piseddu, G, Schinco, Pc, Messina, M, Rossetti, G, Barillari, G, Feola, G, Franchini, M, Gandini, G, Castaman, G, Rodeghiero, F, and Italian Association of Haemophilia, Centres

Subjects
Clotting factor, Male, Pediatrics, medicine.medical_specialty, Factor VIII, business.industry, Haemophilia A, Hematology, Disease, medicine.disease, Haemophilia, Hemophilia A, Hemophilia B, Factor IX, On demand treatment, Coagulation, Italy, Surveys and Questionnaires, medicine, Humans, Female, National registry, business, Coagulation Disorder
Abstract

Dear Sir, Treatment of haemophilia is costly, and it must be carefully monitored by physicians and health authorities, so as to improve the planning of the supply of factor concentrates obtained from the fractionation of human plasma or by recombinant technology. Therapy with coagulation factors can be used either prophylactically to prevent bleeding, which entails multiple weekly infusions, or on-demand, through infusion of the amount of factors determined by the severity of the disease and patient’s weight, to stop ongoing bleeding. According to a recent study, factor VIII (FVIII) consumption among persons with haemophilia A (HA) has increased in countries with high-income economies (Stonebraker et al., 2010). In Italy, haemophiliacs and other persons with congenital coagulation disorders are monitored by the National Registry of Congenital Coagulopathies (NRCC) established at the Istituto Superi=

Published
2011

5. Principles of treatment and update of recommendations for the management of haemophilia and congenital bleeding disorders in Italy

Author

Rocino, A., Coppola, A., Franchini, M., Castaman, G., Santoro, C., Zanon, E., Santagostino, E., Morfini, M., Accorsi, A., Brigida Aru, A., Biasoli, C., Cantori, I., Cesaro, S., Ciabatta, C., De Cristofaro, R., Delios, G., Di Minno, G., D'Incà, M., Dragani, A., Pietro Ettorre, C., Gagliano, F., Gamba, G., Gandini, G., Giordano, P., Giuffrida, G., Gresele, P., Jane Hassan, H., Latella, C., Luciani, M., Margaglione, M., Marietta, M., Gabriella Mazzucconi, M., Messina, M., Claudio Molinari, A., Dora Notarangelo, L., Peyvandi, F., Piseddu, G., Rossetti, G., Rossi, V., Carlotta Santoro, R., Schiavoni, M., Schinco, P., Luisa Serino, M., Tagliaferri, A., Testa, S., Rocino, A, Coppola, A, Franchini, M, Castaman, G, Santoro, C, Zanon, E, Santagostino, E, Morfini, M, and DI MINNO, Giovanni

Subjects
therapy, Settore MED/09 - Medicina Interna, congenital bleeding disorders, haemophilia with inhibitors, haemophilia, prophylaxis, Hematology, Immunology and Allergy, Recommendation
Published
2014

6. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis

Author

Iorio, A, Oliovecchio, E, Morfini, M, Mannucci, Pm, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdré, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Molinari, Ac, Boeri, E, Caprino, D, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Federici, Ab, Marietta, M, Pedrazzoli, P, Di Minno, G, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, Hassan, J, D'Inca, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Maria Gabriella, Santoro, Cristina, Piseddu, G, Carla Schinco, P, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Franchini, M, Castaman, G., Iorio, A, Oliovecchio, E, Morfini, M, Mannucci, Pm, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdré, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Molinari, Ac, Boeri, E, Caprino, D, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Federici, Ab, Marietta, M, Pedrazzoli, P, DI MINNO, Giovanni, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, Hassan, J, D'Inca, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Mg, Santoro, C, Piseddu, G, Carla Schinco, P, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Franchini, M, and Castaman, G.

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Adolescent, Haemophilia A, MEDLINE, Haemophilia, Severity of Illness Index, haemophilia, registry, von willebrand's disease, von willebrand’s disease, Blood Coagulation Disorders, Inherited, Von willebrand, Severity of illness, Health care, Computer software, medicine, Humans, Haemophilia B, Registries, Age of Onset, Child, Genetics (clinical), Societies, Medical, Aged, business.industry, Infant, Hematology, General Medicine, Middle Aged, medicine.disease, Databases as Topic, Italy, Child, Preschool, Data Interpretation, Statistical, Female, Medical emergency, business
Abstract

National haemophilia registries are powerful instruments to support health care and research. A national registry was established in Italy by the Ministry of Health until 1999. Since 2003 the Italian Association of Haemophilia Centres (AICE) started a new programme aiming at building up the Italian Registry of Haemophilia and Allied Disorders. The AICE identified an expert panel to steer the registry. A computer software to assist patient management was developed and all the AICE-affiliated haemophilia treatment centres (HTC) were prompted to adopt it. Twice a year a predefined set of anonymized data is centralized and merged into a national database. Duplicated entries are managed through a confidentiality sparing mechanism. The database covers sociodemographic, clinical, laboratory and treatment data. A subset of data are shared with the Ministry of Health (Istituto Superiore di Sanita,ISS).Overall, data were collected six times by 43 of 49 HTC; 41 centres updated their patients' records up to December 2006. The database contains 6632 unique records, 442 of them referring to dead patients. Database growth and missing data clearance showed a constantly positive trend over time. The database has collected records of the following alive patients - haemophilia A: 1364 severe, 398 moderate and 935 mild; haemophilia B: 231 severe, 138 moderate and 204 mild; von Willebrand's disease: 1208 type 1, 346 type 2 and 96 type 3. Inhibitor patients were 296 (of which 194 high responders and 65 low responders).The Italian registry run by AICE adds to the list of the available national haemophilia registries and is intended to establish treatment guidelines and foster research projects in Italy.

Published
2008

7. Non-genetic risk factors and the development of inhibitors in haemophilia: a comprehensive review and consensus report.

Author

ASTERMARK, J., ALTISENT, C., BATOROVA, A., DINIZ, M. J., GRINGERI, A., HOLME, P. A., KARAFOULIDOU, A., LOPEZ-FERNÁNDEZ, M. F., REIPERT, B. M., ROCINO, A., SCHIAVONI, M., von DEPKA, M., WINDYGA, J., and FIJNVANDRAAT, K.

Subjects
HEMOPHILIA, BLOOD coagulation disorders, VACCINATION, PREGNANCY, BLOOD diseases, ENZYME inhibitors
Abstract

The development of inhibitors to the infused factor in patients with haemophilia is a serious clinical problem. Recent evidence suggests that alongside the strong genetic contribution to inhibitor formation, there are a number of non-genetic factors – perceived by the immune system as danger signals – which promote formation of inhibitors. This study provides a comprehensive review of clinical studies relating to these factors and also presents a survey of opinion concerning their importance and clinical influence, conducted among the members of the European Haemophilia Treatment Standardisation Board (EHTSB). Taken together, this information highlights the lack of robust data concerning the influence of several non-genetic risk factors on inhibitor development, and an urgent need for prospective, well-conducted studies that adhere to recommendations made by the European Medicines Agency (EMEA) for studying inhibitors. Based on current literature, the EHTSB formulated consensus recommendations. It is desirable to minimize intensive treatment wherever possible, given the clinical situation. Prophylaxis should be offered to all children, although we still need to determine optimal dosing with respect to inhibitor development, and age for starting treatment. Vaccinations should be given subcutaneously and concomitant factor concentrate infusions avoided. According to the board, there is no evidence in the literature supporting suggestions that the type of concentrate influences inhibitor risk; but all patients should be monitored during their first exposures. Furthermore, there is no evidence to support an association between pregnancy-related issues, breast feeding and treatment-related factors (e.g. route of administration, or use of blood components) and inhibitor development. [ABSTRACT FROM AUTHOR]

Published
2010
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8. Current use of by-passing agents in Europe in the management of acute bleeds in patients with haemophilia and inhibitors.

Author

ASTERMARK, J., ROCINO, A., VON DEPKA, M., VAN DEN BERG, H. M., GRINGERI, A., MANTOVANI, L. G., MORADO, M., GARRIDO, R. P., SCHIAVONI, M., VILLAR, A., and WINDYGA, J.

Subjects
HEMOPHILIACS, CHEMICAL inhibitors, IMMUNOLOGICAL tolerance, HEMORRHAGE, PROTHROMBIN, PATIENT monitoring
Abstract

The ultimate goal of treatment for patients with inhibitory antibodies should be to permanently eradicate the inhibitor by immune tolerance induction therapy (ITI). However, ITI procedures fail in a substantial number of patients and in many countries ITI is not even offered owing to its high cost. How patients with inhibitors are managed in different European countries is evaluated with a special focus on the use of by-passing agents, i.e. recombinant FVIIa (rFVIIa) and activated prothrombin complex concentrates (aPCC), as well as the type of monitoring performed. Investigators from 22 large haemophilia centres participating within the network of the European Haemophilia Therapy Standardisation Board (EHTSB) were asked to complete a questionnaire. rFVIIa was routinely used in all centres for both children and adults at dosages ranging from 90 to 250 μg kg−1 at an interval of 2–4 h. aPCC was used in 85% of the centres in adults and in 25% of the centres in children with haemophilia A at dosages of 50–100 IU kg−1 every 6–12 h. The corresponding figures for children and adults with haemophilia B were 40% and 15% of the centres, respectively. Higher dosages of both agents were considered in the case of life-threatening bleeds. General recommendations were developed, based on the information provided by the survey. The results clearly indicate the need for well-designed comparative studies to optimize the use of by-passing agents. [ABSTRACT FROM AUTHOR]

Published
2007
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9. Current European practice in immune tolerance induction therapy in patients with haemophilia and inhibitors.

Author

Astermark, J., Morado, M., Rocino, A., Van Den Berg, H. M., Von Depka, M., Gringeri, A., Mantovani, L., Garrido, R. P., Schiavoni, M., Villar, A., and Windyga, J.

Subjects
HEMOPHILIA, IMMUNOLOGICAL tolerance, BLOOD diseases, BLOOD coagulation disorders, IMMUNE response
Abstract

The management of patients with inhibitors is an important challenge in haemophilia care. The lack of randomized controlled trials means that clinical decisions are generally based on subjective opinions, and purchasers’ attention is likely to focus on the costs of treatment. In order to assess the current management of inhibitor patients and use of immune tolerance induction therapy (ITI) in Europe, we performed a survey within a European network of 21 comprehensive care centres from 14 countries (the European Haemophilia Therapy Standardisation Board). The survey identified a total of 381 patients with inhibitors attending the centres, 211 (55.4%) of whom had never been exposed to ITI. Between 1998 and 2003, the centres performed 233 procedures and 114 (48.9%) were successful. The survey demonstrated that dosing, which is the time to start and stop the ITI, the type of concentrate to use and the definition of success varied among the centres. Well-designed trials are warranted to guide decision-making, but in the absence of these studies we have developed consensus guidance for the management of inhibitor patients based on current clinical practice, as identified by the survey, and review of the literature. [ABSTRACT FROM AUTHOR]

Published
2006
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