INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORT

Case Report: Hemophilia A is a hereditary bleeding disorder due to factor VIII deficiency. With the advances in the treatment of hemophilia in recent years, the average life expectancy of patients has reached the healthy population. Along with prolonged life, additional diseases and intervention req...

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Main Authors: Ferda Can, Gaye Kalacı, Ozge Kösemehmetoğlu, Davut Kamacı, Sema Akıncı, Sule Mine Bakanay Öztürk, Imdat Dilek, Tekin Güney
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:Hematology, Transfusion and Cell Therapy
Online Access:http://www.sciencedirect.com/science/article/pii/S2531137924028876
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author Ferda Can
Gaye Kalacı
Ozge Kösemehmetoğlu
Davut Kamacı
Sema Akıncı
Sule Mine Bakanay Öztürk
Imdat Dilek
Tekin Güney
author_facet Ferda Can
Gaye Kalacı
Ozge Kösemehmetoğlu
Davut Kamacı
Sema Akıncı
Sule Mine Bakanay Öztürk
Imdat Dilek
Tekin Güney
author_sort Ferda Can
collection DOAJ
description Case Report: Hemophilia A is a hereditary bleeding disorder due to factor VIII deficiency. With the advances in the treatment of hemophilia in recent years, the average life expectancy of patients has reached the healthy population. Along with prolonged life, additional diseases and intervention requirements are developing in this patient group. Due to the developments, management of patients going under interventions are more clear and easier. In this case, a patient who underwent an intervention with extended half-life factor therapy was presented.Forty-three-year-old male patient with severe hemophilia A was evaluated on request for circumcision surgery while using prophylactically extended half-life factor therapy 2 × 1000 Units / week. Tranexamic acid was started one day preoperatively to the patient whose basal factor level was below 1% and whose inhibitory level was negative. Body weight of the patient was 63 kg. Extended half-life factor VIII preparation (efmorogtocog alfa) loading dose of 3000 units was administered befrore half an hour of the procedure. aPtt was detected for 30 seconds and factor VIII level was 55% 30 minutes after loading dose. The patient was given appropriate sedative treatment to prevent pre-perioperative erection. The operation was carried out without any problems. 1500 Units 12 hours after the loading dose, and 24 hours after this dose was performed. The patient was discharged without complications without bleeding. Factor therapy was continued with prophylaxis dosing. Tranexamic acid was continued for 7 days. No complications were observed.Interventional procedures of hemophilia patients can be performed without complications with a multidisciplinary approach under appropriate dose and scheme factor therapy. In the case, an interventinal procedure was made by giving an extended half-life factor to a severe hemophilia patient who could not have a circumcision operation for many years due to previous hesitations of both patient and surgeons.
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issn 2531-1379
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publishDate 2024-12-01
publisher Elsevier
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series Hematology, Transfusion and Cell Therapy
spelling doaj-art-e7c93bed513641599b0680e6a76b4c682024-12-26T08:57:05ZengElsevierHematology, Transfusion and Cell Therapy2531-13792024-12-0146S55INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORTFerda Can0Gaye Kalacı1Ozge Kösemehmetoğlu2Davut Kamacı3Sema Akıncı4Sule Mine Bakanay Öztürk5Imdat Dilek6Tekin Güney7Ankara Bilkent Şehir Hastanesi; Corresponding author.Ankara Bilkent Şehir HastanesiAnkara Bilkent Şehir HastanesiAnkara Bilkent Şehir HastanesiAnkara Bilkent Şehir HastanesiAnkara Bilkent Şehir HastanesiAnkara Bilkent Şehir HastanesiAnkara Bilkent Şehir HastanesiCase Report: Hemophilia A is a hereditary bleeding disorder due to factor VIII deficiency. With the advances in the treatment of hemophilia in recent years, the average life expectancy of patients has reached the healthy population. Along with prolonged life, additional diseases and intervention requirements are developing in this patient group. Due to the developments, management of patients going under interventions are more clear and easier. In this case, a patient who underwent an intervention with extended half-life factor therapy was presented.Forty-three-year-old male patient with severe hemophilia A was evaluated on request for circumcision surgery while using prophylactically extended half-life factor therapy 2 × 1000 Units / week. Tranexamic acid was started one day preoperatively to the patient whose basal factor level was below 1% and whose inhibitory level was negative. Body weight of the patient was 63 kg. Extended half-life factor VIII preparation (efmorogtocog alfa) loading dose of 3000 units was administered befrore half an hour of the procedure. aPtt was detected for 30 seconds and factor VIII level was 55% 30 minutes after loading dose. The patient was given appropriate sedative treatment to prevent pre-perioperative erection. The operation was carried out without any problems. 1500 Units 12 hours after the loading dose, and 24 hours after this dose was performed. The patient was discharged without complications without bleeding. Factor therapy was continued with prophylaxis dosing. Tranexamic acid was continued for 7 days. No complications were observed.Interventional procedures of hemophilia patients can be performed without complications with a multidisciplinary approach under appropriate dose and scheme factor therapy. In the case, an interventinal procedure was made by giving an extended half-life factor to a severe hemophilia patient who could not have a circumcision operation for many years due to previous hesitations of both patient and surgeons.http://www.sciencedirect.com/science/article/pii/S2531137924028876
spellingShingle Ferda Can
Gaye Kalacı
Ozge Kösemehmetoğlu
Davut Kamacı
Sema Akıncı
Sule Mine Bakanay Öztürk
Imdat Dilek
Tekin Güney
INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORT
Hematology, Transfusion and Cell Therapy
title INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORT
title_full INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORT
title_fullStr INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORT
title_full_unstemmed INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORT
title_short INTERVENTIONAL PROCEDURE IN HEMOPHILIA A PATIENT WITH EXTENDED HALF-LIFE FACTOR THERAPY- CIRCUMCISION- CASE REPORT
title_sort interventional procedure in hemophilia a patient with extended half life factor therapy circumcision case report
url http://www.sciencedirect.com/science/article/pii/S2531137924028876
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