A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults
Acquired hemophilia A (AHA) is a rare bleeding disorder, especially in adolescents and young adults (AYAs) attributable to the development of autoantibodies against coagulation factor VIII (FVIII). AHA diagnosis is difficult; patients lack any history of coagulopathy. We report here on an AYA with A...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
The Korean Society of Pediatric Hematology-Oncology
2022-04-01
|
| Series: | Clinical Pediatric Hematology-Oncology |
| Subjects: | |
| Online Access: | http://journaleditor.inforang.com/journal/view.html?doi=10.15264/cpho.2022.29.1.21 |
| _version_ | 1818149958725926912 |
|---|---|
| author | Min Jeong Lee Young Shil Park |
| author_facet | Min Jeong Lee Young Shil Park |
| author_sort | Min Jeong Lee |
| collection | DOAJ |
| description | Acquired hemophilia A (AHA) is a rare bleeding disorder, especially in adolescents and young adults (AYAs) attributable to the development of autoantibodies against coagulation factor VIII (FVIII). AHA diagnosis is difficult; patients lack any history of coagulopathy. We report here on an AYA with AHA who responded well to treatment. A 19-year-old woman visited our hospital with painful swelling of the right lower leg. She had no past or familial history of a bleeding disorder. The initial laboratory data revealed a prolonged activated partial thromboplastin time and an uncorrected mixing test result. The FVIII activity was below 1% and the FVIII antibody level 22.4 Bethesda units. She was diagnosed with AHA and treated with recombinant activated coagulation factor VII, activated prothrombin complex concentrates and an oral steroid. After 9 months, FVIII antibody level was negative and the FVIII activity was normalized. AHA is very rare especially in AYAs, but physicians must be suspicious about the disorder and plan specialized coagulation tests to diagnose the disease. An early diagnosis of acquired bleeding disorders should be done for initiating the adequate treatment immediately by both controlling the acute bleeding episode and eliminating FVIII antibodies. |
| first_indexed | 2024-12-11T13:15:18Z |
| format | Article |
| id | doaj.art-97eaf202911b44a99caf5ca970d7573a |
| institution | Directory Open Access Journal |
| issn | 2233-5250 |
| language | English |
| last_indexed | 2024-12-11T13:15:18Z |
| publishDate | 2022-04-01 |
| publisher | The Korean Society of Pediatric Hematology-Oncology |
| record_format | Article |
| series | Clinical Pediatric Hematology-Oncology |
| spelling | doaj.art-97eaf202911b44a99caf5ca970d7573a2022-12-22T01:06:04ZengThe Korean Society of Pediatric Hematology-OncologyClinical Pediatric Hematology-Oncology2233-52502022-04-01291212410.15264/cpho.2022.29.1.21cpho.2022.29.1.21A Rare Case of Acquired Hemophilia A in Adolescents and Young AdultsMin Jeong Lee0Young Shil Park1Department of Pediatrics, Kyung Hee University Hospital at Gangdong, Seoul, KoreaDepartment of Pediatrics, Kyung Hee University Hospital at Gangdong, Seoul, KoreaAcquired hemophilia A (AHA) is a rare bleeding disorder, especially in adolescents and young adults (AYAs) attributable to the development of autoantibodies against coagulation factor VIII (FVIII). AHA diagnosis is difficult; patients lack any history of coagulopathy. We report here on an AYA with AHA who responded well to treatment. A 19-year-old woman visited our hospital with painful swelling of the right lower leg. She had no past or familial history of a bleeding disorder. The initial laboratory data revealed a prolonged activated partial thromboplastin time and an uncorrected mixing test result. The FVIII activity was below 1% and the FVIII antibody level 22.4 Bethesda units. She was diagnosed with AHA and treated with recombinant activated coagulation factor VII, activated prothrombin complex concentrates and an oral steroid. After 9 months, FVIII antibody level was negative and the FVIII activity was normalized. AHA is very rare especially in AYAs, but physicians must be suspicious about the disorder and plan specialized coagulation tests to diagnose the disease. An early diagnosis of acquired bleeding disorders should be done for initiating the adequate treatment immediately by both controlling the acute bleeding episode and eliminating FVIII antibodies.http://journaleditor.inforang.com/journal/view.html?doi=10.15264/cpho.2022.29.1.21hemophilia aacquired hemophilia acoagulation factor viiiautoantibodiesimmunosuppressive treatment |
| spellingShingle | Min Jeong Lee Young Shil Park A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults Clinical Pediatric Hematology-Oncology hemophilia a acquired hemophilia a coagulation factor viii autoantibodies immunosuppressive treatment |
| title | A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults |
| title_full | A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults |
| title_fullStr | A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults |
| title_full_unstemmed | A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults |
| title_short | A Rare Case of Acquired Hemophilia A in Adolescents and Young Adults |
| title_sort | rare case of acquired hemophilia a in adolescents and young adults |
| topic | hemophilia a acquired hemophilia a coagulation factor viii autoantibodies immunosuppressive treatment |
| url | http://journaleditor.inforang.com/journal/view.html?doi=10.15264/cpho.2022.29.1.21 |
| work_keys_str_mv | AT minjeonglee ararecaseofacquiredhemophiliaainadolescentsandyoungadults AT youngshilpark ararecaseofacquiredhemophiliaainadolescentsandyoungadults AT minjeonglee rarecaseofacquiredhemophiliaainadolescentsandyoungadults AT youngshilpark rarecaseofacquiredhemophiliaainadolescentsandyoungadults |