Hémophilie A acquise : caractéristiques clinico-biologiques et prise en charge thérapeutique d’une série de huit patients hospitalisés à Lariboisière et Saint-Louis (notice n° 275695)
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fixed length control field | 03319cam a2200361 4500500 |
005 - DATE AND TIME OF LATEST TRANSACTION | |
control field | 20250117193439.0 |
041 ## - LANGUAGE CODE | |
Language code of text/sound track or separate title | fre |
042 ## - AUTHENTICATION CODE | |
Authentication code | dc |
100 10 - MAIN ENTRY--PERSONAL NAME | |
Personal name | Noizat, Clara |
Relator term | author |
245 00 - TITLE STATEMENT | |
Title | Hémophilie A acquise : caractéristiques clinico-biologiques et prise en charge thérapeutique d’une série de huit patients hospitalisés à Lariboisière et Saint-Louis |
260 ## - PUBLICATION, DISTRIBUTION, ETC. | |
Date of publication, distribution, etc. | 2021.<br/> |
500 ## - GENERAL NOTE | |
General note | 7 |
520 ## - SUMMARY, ETC. | |
Summary, etc. | RésuméL’hémophilie A acquise est une atteinte auto-immune rare, liée à l’apparition d’auto-anticorps dirigés contre le facteur VIII circulant, et caractérisée par un syndrome hémorragique majeur. L’hémophilie A acquise est une urgence médicale diagnostique et thérapeutique pouvant engager le pronostic vital. Nous décrivons ici les caractéristiques clinico-biologiques et thérapeutiques d’une série de huit patients atteints d’hémophilie A acquise pris en charge entre 2015 et 2020 dans les centres hospitalo-universitaires Lariboisière et Saint-Louis (Paris, France). Devant toute suspicion clinique et/ou biologique d’hémophilie A acquise, la prise en charge des patients doit être faite sans délai et repose sur trois axes publiés selon des études de cohortes multicentriques essentiellement observationnelles : le traitement symptomatique du syndrome hémorragique, le traitement immunosuppresseur d’éradication des auto-anticorps anti-facteur VIII avec la gestion de leurs possibles complications, ainsi que le traitement étiologique de la pathologie sous-jacente pour les formes secondaires. |
520 ## - SUMMARY, ETC. | |
Summary, etc. | Acquired hemophilia A is a rare autoimmune disease, linked to the appearance of autoantibodies directed against circulating factor VIII, and characterized by a major hemorrhagic syndrome. Acquired hemophilia A is a life-threatening diagnostic and therapeutic medical emergency. We describe here the cohort of patients with acquired hemophilia A treated between 2015 and 2020 at Lariboisière and Saint-Louis University Hospitals (Paris, France). We remind you here of the measures to be taken without delay in the face of any clinical and/or biological suspicion. Management is based on three main areas published in multicentre cohort studies, essentially observational: symptomatic treatment to control the hemorrhagic syndrome, immunosuppressive treatment to eradicate autoantibodies and manage their possible complications, and etiological treatment of the underlying pathology for secondary forms. |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | agent hémostatique |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | hémophilie A acquise |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | hémorragie |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | immunosuppresseur |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | anticorps anti-facteur VIII |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | haemostatic agent |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | bleeding |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | immunosuppressive therapy |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | acquired haemophilia A |
690 ## - LOCAL SUBJECT ADDED ENTRY--TOPICAL TERM (OCLC, RLIN) | |
Topical term or geographic name as entry element | FVIII autoantibodies |
700 10 - ADDED ENTRY--PERSONAL NAME | |
Personal name | Béranger, Nicolas |
Relator term | author |
700 10 - ADDED ENTRY--PERSONAL NAME | |
Personal name | Joly, Bérangère S. |
Relator term | author |
700 10 - ADDED ENTRY--PERSONAL NAME | |
Personal name | Veyradier, Agnès |
Relator term | author |
700 10 - ADDED ENTRY--PERSONAL NAME | |
Personal name | Stepanian, Alain |
Relator term | author |
700 10 - ADDED ENTRY--PERSONAL NAME | |
Personal name | Siguret, Virginie |
Relator term | author |
700 10 - ADDED ENTRY--PERSONAL NAME | |
Personal name | Itzhar-Baikian, Nathalie |
Relator term | author |
786 0# - DATA SOURCE ENTRY | |
Note | Annales de Biologie Clinique | 79 | 1 | 2021-01-01 | p. 75-82 | 0003-3898 |
856 41 - ELECTRONIC LOCATION AND ACCESS | |
Uniform Resource Identifier | <a href="https://shs.cairn.info/revue-annales-de-biologie-clinique-2021-1-page-75?lang=fr&redirect-ssocas=7080">https://shs.cairn.info/revue-annales-de-biologie-clinique-2021-1-page-75?lang=fr&redirect-ssocas=7080</a> |
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