Management of a patient with factor X deficiency with FEIBA: a case report
Hereditary factor X (FX) deficiency is a rare autosomal recessive disorder that increases bleeding tendencies, ranging from epistaxis to intracranial haemorrhage (ICH), which can be life-threatening. Single factor concentrates are recommended for treating rare bleeding disorders; however, in India m...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Sciendo
2023-08-01
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| Series: | The Journal of Haemophilia Practice |
| Subjects: | |
| Online Access: | https://doi.org/10.2478/jhp-2023-0012 |
| _version_ | 1797354360831737856 |
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| author | Borboruah Luish Dutta Anupam |
| author_facet | Borboruah Luish Dutta Anupam |
| author_sort | Borboruah Luish |
| collection | DOAJ |
| description | Hereditary factor X (FX) deficiency is a rare autosomal recessive disorder that increases bleeding tendencies, ranging from epistaxis to intracranial haemorrhage (ICH), which can be life-threatening. Single factor concentrates are recommended for treating rare bleeding disorders; however, in India most people with FX deficiency are treated with fresh frozen plasma (FFP). We report a case of FX deficiency in a child with a history of intracranial bleeding who was found to have a novel mutation in the F10 gene. Although managed with weekly prophylactic FFP infusions, he continued to experience regular bleeding including two further instances of ICH. Other therapeutic options were unavailable or unaffordable. When the Indian Government added FEIBA to the essential drug list, a decision was made to try FEIBA prophylaxis to better manage his bleeding. In 2019, he was started on a weekly dose of FEIBA, 500 IU (20 IU/ kg and his prophylactic FFP transfusion regimen was stopped. His bleeding episodes started to reduce after two months of starting FEIBA prophylaxis. Over the last three years he has had only four minor bleeding episodes and has remained completely free of major bleeding. He is now able to receive home-based therapy and his prognosis can be considered to be improved. FEIBA may be a useful medicinal therapy for FX-deficient patients who suffer severe haemorrhagic episodes in countries where plasma-derived factor X (pdFX) is not available. |
| first_indexed | 2024-03-08T13:48:21Z |
| format | Article |
| id | doaj.art-7272ec6bebcf4693a715acf70b96dee9 |
| institution | Directory Open Access Journal |
| issn | 2055-3390 |
| language | English |
| last_indexed | 2024-03-08T13:48:21Z |
| publishDate | 2023-08-01 |
| publisher | Sciendo |
| record_format | Article |
| series | The Journal of Haemophilia Practice |
| spelling | doaj.art-7272ec6bebcf4693a715acf70b96dee92024-01-16T07:20:03ZengSciendoThe Journal of Haemophilia Practice2055-33902023-08-01101687310.2478/jhp-2023-0012Management of a patient with factor X deficiency with FEIBA: a case reportBorboruah Luish0Dutta Anupam1Assam Medical College and Hospital, Dibrugarh, IndiaAssam Medical College and Hospital, Dibrugarh, IndiaHereditary factor X (FX) deficiency is a rare autosomal recessive disorder that increases bleeding tendencies, ranging from epistaxis to intracranial haemorrhage (ICH), which can be life-threatening. Single factor concentrates are recommended for treating rare bleeding disorders; however, in India most people with FX deficiency are treated with fresh frozen plasma (FFP). We report a case of FX deficiency in a child with a history of intracranial bleeding who was found to have a novel mutation in the F10 gene. Although managed with weekly prophylactic FFP infusions, he continued to experience regular bleeding including two further instances of ICH. Other therapeutic options were unavailable or unaffordable. When the Indian Government added FEIBA to the essential drug list, a decision was made to try FEIBA prophylaxis to better manage his bleeding. In 2019, he was started on a weekly dose of FEIBA, 500 IU (20 IU/ kg and his prophylactic FFP transfusion regimen was stopped. His bleeding episodes started to reduce after two months of starting FEIBA prophylaxis. Over the last three years he has had only four minor bleeding episodes and has remained completely free of major bleeding. He is now able to receive home-based therapy and his prognosis can be considered to be improved. FEIBA may be a useful medicinal therapy for FX-deficient patients who suffer severe haemorrhagic episodes in countries where plasma-derived factor X (pdFX) is not available.https://doi.org/10.2478/jhp-2023-0012factor x deficiencyfresh frozen plasma (ffp)feibaintracranial haemorrhagecase report |
| spellingShingle | Borboruah Luish Dutta Anupam Management of a patient with factor X deficiency with FEIBA: a case report The Journal of Haemophilia Practice factor x deficiency fresh frozen plasma (ffp) feiba intracranial haemorrhage case report |
| title | Management of a patient with factor X deficiency with FEIBA: a case report |
| title_full | Management of a patient with factor X deficiency with FEIBA: a case report |
| title_fullStr | Management of a patient with factor X deficiency with FEIBA: a case report |
| title_full_unstemmed | Management of a patient with factor X deficiency with FEIBA: a case report |
| title_short | Management of a patient with factor X deficiency with FEIBA: a case report |
| title_sort | management of a patient with factor x deficiency with feiba a case report |
| topic | factor x deficiency fresh frozen plasma (ffp) feiba intracranial haemorrhage case report |
| url | https://doi.org/10.2478/jhp-2023-0012 |
| work_keys_str_mv | AT borboruahluish managementofapatientwithfactorxdeficiencywithfeibaacasereport AT duttaanupam managementofapatientwithfactorxdeficiencywithfeibaacasereport |