| H01759 | |
| H番号 | H01759 |
| 名称 | 自己免疫性後天性凝固第13因子欠乏症; 自己免疫性出血病 XIII |
| 概要 | Autoimmune acquired factor XIII (F13) deficiency or autoimmune hemorrhaphilia resulted from the generation of anti-F13 antibodies (AHFXIII) is a rare but severe life-threatening bleeding disorder. In contrast, non-autoimmune hemorrhagic acquired F13 deficiency (HAF13D) is a less severe bleeding disease secondary to various disease conditions. AHFXIII must be distinguished from HAF13D since AHFXIII requires immunosuppressive therapy to eradicate autoantibodies in addition to F13 replacement therapy to arrest bleeding. Patients with acquired F13 deficiency, in particular those with AHFXIII, are on the increase, at least in Japan. |
| カテゴリ | 血液疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | - |
| コメント | - |
| リンク | ICD-11: 3B21 ICD-10: D68.4 MeSH: D005177 |
| 文献 | PMID:27542511 著者 Ichinose A タイトル Autoimmune acquired factor XIII deficiency due to anti-factor XIII/13 antibodies: A summary of 93 patients. 雑誌 Blood Rev 31:37-45 (2017) DOI:10.1016/j.blre.2016.08.002 PMID:24476525 著者 Tahlan A, Ahluwalia J タイトル Factor XIII: congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit. 雑誌 Arch Pathol Lab Med 138:278-81 (2014) DOI:10.5858/arpa.2012-0639-RS PMID:22477542 著者 Ichinose A タイトル Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control. 雑誌 Int J Hematol 95:362-70 (2012) DOI:10.1007/s12185-012-1064-3 |