Haemophilia pseudotumours in patients with inhibitors.
| Autor: | Caviglia H; Department of Orthopaedic Surgery and Traumatology, Dr. Juan A. Fernández General Hospital, Buenos Aires, Argentina.; Haemophilia Foundation, Buenos Aires, Argentina., Candela M; Haemophilia Foundation, Buenos Aires, Argentina., Landro ME; Department of Orthopaedic Surgery and Traumatology, Dr. Juan A. Fernández General Hospital, Buenos Aires, Argentina., Douglas Price AL; Department of Orthopaedic Surgery and Traumatology, Dr. Juan A. Fernández General Hospital, Buenos Aires, Argentina., Neme D; Haemophilia Foundation, Buenos Aires, Argentina., Galatro GA; Department of Orthopaedic Surgery and Traumatology, Dr. Juan A. Fernández General Hospital, Buenos Aires, Argentina.; Haemophilia Foundation, Buenos Aires, Argentina. |
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| Jazyk: | angličtina |
| Zdroj: | Haemophilia : the official journal of the World Federation of Hemophilia [Haemophilia] 2015 Sep; Vol. 21 (5), pp. 681-5. Date of Electronic Publication: 2015 Feb 16. |
| DOI: | 10.1111/hae.12632 |
| Abstrakt: | Development of inhibitors against factor VIII (FVIII) or FIX is the most serious complication of replacement therapy in patients with haemophilia. Haemophilic pseudotumours in a patient with inhibitors can lead to devastating consequences. The aim of this study is to show our experience in the treatment of 10 pseudotumours in 7 patients with inhibitors who were treated by the same multidisciplinary team in the period between January 2000 and March 2013. Seven severe haemophilia A patients were treated at the Haemophilia Foundation in Buenos Aires, Argentina, for 10 pseudotumours. Eight were bone pseudotumours and two soft tissue. All patients underwent imaging studies at baseline to assess the size and content of the lesion. The patients received Buenos Aires protocol as conservative treatment of their pseudotumours for 6 weeks, after which they were evaluated. Only one patient responded to conservative treatment. Surgery was performed on the others six patients, since their pseudotumours did not shrink to less than half their original size. Any bleeding in the musculoskeletal system must be treated promptly in order to prevent pseudotumours. When pseudotumours do appear in inhibitor patients, they can be surgically removed when patients received proper haemostatic coverage, improving the quality of life of these patients. (© 2015 John Wiley & Sons Ltd.) |
| Databáze: | MEDLINE |
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