<abstract xmlns="http://www.w3.org/1999/xhtml">The development of factor VIII inhibitors (allo-antibodies) continues to be a major complication in the management of severe forms of hemophilia A, especially as far as treatment and treatment response monitoring is concerned. The need to implement a reliable laboratory assay is all the more obvious if major surgery occurs, when conventional tests (activated partial thromboplastin time APTT, prothrombin time PT, factor VIII level) are of no avail and there is a very fragile balance between bleeding and thrombosis.We report the case of a 32 year-old patient diagnosed with severe Hemophilia A, referred to the Comprehensive Center for the Diagnosis and Treatment of Hemophilia of the Fundeni Clinical Institute for a multidisciplinary assessment in view of a total left hip arthroplasty due to aseptic necrosis of the femoral neck.Workup showed a high inhibitor titer (>200 BU). Taking into consideration the interindividual variability of the response to bypassing agents, as well as the bleeding risk associated with a major orthopedic surgery, we used thromboelastography (TEG) to assess the patient’s response to aPCC (activated prothrombin complex concentrate) and rFVIIa (activated recombinant factor VII). The findings helped select the optimal replacement scheme to ensure perioperative hemostasis.</abstract>

The development of factor VIII inhibitors (allo-antibodies) continues to be a major complication in the management of severe forms of hemophilia A, especially as far as treatment and treatment response monitoring is concerned. The need to implement a reliable laboratory assay is all the more obvious if major surgery occurs, when conventional tests (activated partial thromboplastin time APTT, prothrombin time PT, factor VIII level) are of no avail and there is a very fragile balance between bleeding and thrombosis.We report the case of a 32 year-old patient diagnosed with severe Hemophilia A, referred to the Comprehensive Center for the Diagnosis and Treatment of Hemophilia of the Fundeni Clinical Institute for a multidisciplinary assessment in view of a total left hip arthroplasty due to aseptic necrosis of the femoral neck.Workup showed a high inhibitor titer (>200 BU). Taking into consideration the interindividual variability of the response to bypassing agents, as well as the bleeding risk associated with a major orthopedic surgery, we used thromboelastography (TEG) to assess the patient’s response to aPCC (activated prothrombin complex concentrate) and rFVIIa (activated recombinant factor VII). The findings helped select the optimal replacement scheme to ensure perioperative hemostasis.

Thromboelastography in pre-surgery monitoring in Hemophilia A with high inhibitor titer: case report and literature review

“Carol Davila” University of Medicine and Pharmacy

Revista Romana de Medicina de Laborator

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

The development of factor VIII inhibitors (allo-antibodies) continues to be a major complication in the management of severe forms of hemophilia A, especially...

Thromboelastography in pre-surgery monitoring in Hemophilia A with high inhibitor titer: case report and literature review

Online veröffentlicht: 04. Mai 2020

Seitenbereich: 217 - 224

Eingereicht: 10. Nov. 2019

Akzeptiert: 05. März 2020

DOI: https://doi.org/10.2478/rrlm-2020-0021

Schlüsselwörter
severe hemophilia A, inhibitors, thromboelastography, bypassing agents

© 2020 Brinza Melen et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Thromboelastography in pre-surgery monitoring in Hemophilia A with high inhibitor titer: case report and literature review

Online veröffentlicht: 04. Mai 2020

Seitenbereich: 217 - 224

Eingereicht: 10. Nov. 2019

Akzeptiert: 05. März 2020

DOI: https://doi.org/10.2478/rrlm-2020-0021

Schlüsselwörtersevere hemophilia A, inhibitors, thromboelastography, bypassing agents

© 2020 Brinza Melen et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Schlüsselwörter
severe hemophilia A, inhibitors, thromboelastography, bypassing agents