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| DOI | 10.1002/HEM3.70111 | ||||
| Año | 2025 | ||||
| Tipo | artículo de investigación |
Citas Totales
Autores Afiliación Chile
Instituciones Chile
% Participación
Internacional
Autores
Afiliación Extranjera
Instituciones
Extranjeras
In a previous paper, a comprehensive clinicopathologic approach to mild and moderate bleeding disorders (MBD) was proposed by an international working group (IWG) as a part of a project promoted by the European Hematology Association (EHA) on the development of guidelines on the various MBDs. A single pre-diagnosis grade 4 bleeding event according to the ISTH-BAT scale or a comparable event after diagnosis was considered sufficient to classify a patient as affected by a severe bleeding disorder (SBD). In this article, the original IWG integrated by experts and patients' representatives proposed by the European Haemophilia Consortium (EHC) and European Association of Haemophilia and Allied Disorders (EAHAD) applied these criteria to mild and moderate hemophilia A and B to establish the proportion of cases that would be reclassified as SBD taking into account bleeding phenotype, thus improving over the current classification based exclusively on basal factor VIII or IX level. To this aim, publications of unselected cases with bleeding history available from birth to the time of publication were considered to estimate the incidence of a first severe bleeding event. More than 20% of cases with mild or moderate hemophilia met the criteria for SBD by experiencing joint or non-joint severe bleeding events. Furthermore, a significant proportion of patients developed an inhibitor against factor VIII or IX. These results, based on a rigorous methodologic approach, substantiate the criticism of the current classification of hemophilia and argue for the adoption of a new classification that takes into account bleeding phenotype in addition to basal clotting activity.
| Ord. | Autor | Género | Institución - País |
|---|---|---|---|
| 1 | Rodeghiero, Francesco | - |
San Bortolo Hosp - Italia
Ospedale San Bortolo - Italia |
| 2 | Ghiotto, Lisanna | - |
San Bortolo Hosp - Italia
Ospedale San Bortolo - Italia |
| 3 | Pontalto, Luca | - |
San Bortolo Hosp - Italia
Ospedale San Bortolo - Italia |
| 4 | Casini, Alessandro | - |
Univ Geneva - Suiza
Hôpitaux universitaires de Genève - Suiza |
| 5 | Castaman, Giancarlo | - |
Careggi Univ Hosp - Italia
Azienda Ospedaliera Careggi - Italia |
| 6 | Abdul-Kadir, Rezan | - |
UCL - Reino Unido
University College London - Reino Unido |
| 7 | Berntorp, Erik | - |
Lund Univ - Suecia
Institutionen för Translationell Medicin - Suecia |
| 8 | Bodo, Imre | - |
Semmelweis Univ - Hungría
Semmelweis Egyetem - Hungría |
| 9 | Degenaar-Dujardin, Manon | - |
European Haemophilia Consortium - Bélgica
|
| 10 | Fijnvandraat, Karin | - |
Univ Amsterdam - Países Bajos
Sanquin Res & Landsteiner Lab - Países Bajos Emma Kinderziekenhuis - Países Bajos Sanquin Research - Países Bajos |
| 11 | Gresele, Paolo | - |
UNIV PERUGIA - Italia
Università degli Studi di Pavia - Italia |
| 12 | Key, Nigel S. | - |
Univ North Carolina - Estados Unidos
UNC School of Medicine - Estados Unidos |
| 13 | Lassila, Riitta | - |
Univ Helsinki - Finlandia
Helsinki University Hospital - Finlandia |
| 14 | Leebeek, Frank W. G. | - |
Erasmus Univ - Países Bajos
Erasmus MC - Países Bajos |
| 15 | Lillicrap, David | - |
Queens Univ - Canadá
Queen's University, School of Medicine - Canadá |
| 16 | Makris, Mike | - |
UNIV SHEFFIELD - Reino Unido
The University of Sheffield - Reino Unido |
| 17 | Meijer, Stephan | - |
Nederlandse Vereniging Hypothecair Planners NVHP - Países Bajos
Nederlandse Vereniging van Hypothecair Planners (NVHP) – for everyone with a congenital bleeding disorder - Países Bajos |
| 18 | Mezzano, Diego | - |
Pontificia Universidad Católica de Chile - Chile
|
| 19 | Noris, Patrizia | - |
Univ Pavia - Italia
Università degli Studi di Pavia - Italia |
| 20 | Pabinger, Ingrid | - |
Med Univ Vienna - Austria
Medizinische Universitat Wien - Austria |
| 21 | Ragni, Margaret V. | - |
Univ Pittsburgh - Estados Unidos
University of Pittsburgh School of Medicine - Estados Unidos |
| 22 | Silva, David | - |
Victoria Eugenia Royal Fdn - España
|
| 23 | Srivastava, Alok | - |
Christian Med Coll & Hosp - India
Christian Medical College, Vellore - India |
| 24 | Tosetto, Alberto | - |
San Bortolo Hosp - Italia
Ospedale San Bortolo - Italia |
| 25 | Windyga, Jerzy | - |
Inst Hematol & Transfus Med - Polonia
Institute of Haematology and Transfusion Medicine, Warsaw - Polonia |
| 26 | Zieger, Barbara | - |
Univ Freiburg - Alemania
Universitätsklinikum Freiburg - Alemania |
| Fuente |
|---|
| Nemzeti Kutatási Fejlesztési és Innovációs Hivatal |
| European Hematology Association |
| European Haemophilia Consortium |
| European Association for Haemophilia and Allied Disorders (EAHAD) |
| NFKI |
| European Association for Haemophilia and Allied Disorders |
| Hematology Project Foundation |
| Agradecimiento |
|---|
| This article is part of a project on inherited mild to moderate bleeding disorders (MBD) endorsed by the European Hematology Association (EHA) in collaboration with the European Association for Haemophilia and Allied Disorders (EAHAD) and the European Haemophilia Consortium (EHC). |
| This study was partially funded by the European Hematology Association (EHA) and the Hematology Project Foundation (Vicenza, Italy). This article is part of a project on inherited mild to moderate bleeding disorders (MBD) endorsed by the European Hematology Association (EHA) in collaboration with the European Association for Haemophilia and Allied Disorders (EAHAD) and the European Haemophilia Consortium (EHC). We thank Claudia Guzzoni for her invaluable secretarial assistance and Lilliana Paganotto for her librarianship service at the Hematology Project Foundation. We thank Attila Szederjesi, whose work was partially supported by grant OTKA-K19_131945 (I. B.) from the Hungarian National Research Development and Innovation Office (NFKI). |
| We thank Claudia Guzzoni for her invaluable secretarial assistance and Lilliana Paganotto for her librarianship service at the Hematology Project Foundation. We thank Attila Szederjesi, whose work was partially supported by grant OTKA\u2010K19_131945 (I. B.) from the Hungarian National Research Development and Innovation Office (NFKI). |