Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A
Acquired hemophilia A (AHA) is a rare bleeding disorder due to acquired antibodies against coagulation factor VIII (FVIII). It is rare in children less than 16 years old, and the incidence is 0.45/million/year. An otherwise healthy, 12-year-old boy was admitted to the ward with a history of swelling...
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Wiley
2018-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2018/6208597 |
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| author | Manori Gamage Sadeepa Weerasinghe Mohamed Nasoor A. M. P. W. Karunarathne Sashi Praba Abeyrathne |
| author_facet | Manori Gamage Sadeepa Weerasinghe Mohamed Nasoor A. M. P. W. Karunarathne Sashi Praba Abeyrathne |
| author_sort | Manori Gamage |
| collection | DOAJ |
| description | Acquired hemophilia A (AHA) is a rare bleeding disorder due to acquired antibodies against coagulation factor VIII (FVIII). It is rare in children less than 16 years old, and the incidence is 0.45/million/year. An otherwise healthy, 12-year-old boy was admitted to the ward with a history of swelling of the right and left forearms, for 1 day duration. He did not have any history of trauma or bleeding disorder. He had prolonged APPTT level with very high antibody titer against factor VIII. His gene expression for factor VIII was found to be normal. He was managed with FEIBA and recombinant FVII activated complexes and prednisolone 1 m/kg/day regime to control bleeding. AHA is associated with several underlying pathologies such as pregnancy, autoimmune diseases, malignancy, medications and infections; however, up to 50% of reported cases are idiopathic. In contrast to congenital haemophilia A, in which haemarthrosis is the hallmark clinical presentation, patients with AHA mainly bleed in to the skin, muscles, and soft tissues. High mortality rate of more than 20% is either to retroperitoneal or intracranial bleeds. Diagnosis is confirmed on isolated prolongation of activated partial thromboplastin time which does not normalize after addition of normal plasma, reducing the factor VIII levels with evidence of FVIII inhibitor activity. They have normal prothrombin time and platelet functions. Management of AHA involves two aspects, namely, eradication of antibodies and maintaining effective haemostasis during a bleeding episode. |
| format | Article |
| id | doaj-art-dae7f4e07f274f678dd8c25fc17871bd |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
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| series | Case Reports in Hematology |
| spelling | doaj-art-dae7f4e07f274f678dd8c25fc17871bd2025-02-03T01:27:53ZengWileyCase Reports in Hematology2090-65602090-65792018-01-01201810.1155/2018/62085976208597Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia AManori Gamage0Sadeepa Weerasinghe1Mohamed Nasoor2A. M. P. W. Karunarathne3Sashi Praba Abeyrathne4Senior Lecturer, Department of Paediatrics, Faculty of Medical Sciences, University of Sri Jayewardenepura, Nugegoda, Sri LankaPaediatric Registrar, University Paediatric Unit, Colombo South Teaching Hospital Kalubowila, Dehiwala-Mount Lavinia, Sri LankaPaediatric Registrar, University Paediatric Unit, Colombo South Teaching Hospital Kalubowila, Dehiwala-Mount Lavinia, Sri LankaSenior Registrar, Department of Pathology, Faculty of Medical Sciences, University of Sri Jayewardenepura, Nugegoda, Sri LankaSenior Registrar, Department of Pathology, Faculty of Medical Sciences, University of Sri Jayewardenepura, Nugegoda, Sri LankaAcquired hemophilia A (AHA) is a rare bleeding disorder due to acquired antibodies against coagulation factor VIII (FVIII). It is rare in children less than 16 years old, and the incidence is 0.45/million/year. An otherwise healthy, 12-year-old boy was admitted to the ward with a history of swelling of the right and left forearms, for 1 day duration. He did not have any history of trauma or bleeding disorder. He had prolonged APPTT level with very high antibody titer against factor VIII. His gene expression for factor VIII was found to be normal. He was managed with FEIBA and recombinant FVII activated complexes and prednisolone 1 m/kg/day regime to control bleeding. AHA is associated with several underlying pathologies such as pregnancy, autoimmune diseases, malignancy, medications and infections; however, up to 50% of reported cases are idiopathic. In contrast to congenital haemophilia A, in which haemarthrosis is the hallmark clinical presentation, patients with AHA mainly bleed in to the skin, muscles, and soft tissues. High mortality rate of more than 20% is either to retroperitoneal or intracranial bleeds. Diagnosis is confirmed on isolated prolongation of activated partial thromboplastin time which does not normalize after addition of normal plasma, reducing the factor VIII levels with evidence of FVIII inhibitor activity. They have normal prothrombin time and platelet functions. Management of AHA involves two aspects, namely, eradication of antibodies and maintaining effective haemostasis during a bleeding episode.http://dx.doi.org/10.1155/2018/6208597 |
| spellingShingle | Manori Gamage Sadeepa Weerasinghe Mohamed Nasoor A. M. P. W. Karunarathne Sashi Praba Abeyrathne Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A Case Reports in Hematology |
| title | Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A |
| title_full | Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A |
| title_fullStr | Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A |
| title_full_unstemmed | Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A |
| title_short | Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A |
| title_sort | progressive intramuscular haematoma in a 12 year old boy a case of acquired haemophilia a |
| url | http://dx.doi.org/10.1155/2018/6208597 |
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