| H01759 | |
| H number | H01759 |
| Name | Autoimmune acquired factor XIII (F13) deficiency; Autoimmune hemorrhaphilia XIII/13 |
| Description | 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. |
| Category | Hematologic disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | - |
| Other DBs | ICD-11: 3B21 ICD-10: D68.4 MeSH: D005177 |
| Reference | PMID:27542511 AUTHORS Ichinose A TITLE Autoimmune acquired factor XIII deficiency due to anti-factor XIII/13 antibodies: A summary of 93 patients. JOURNAL Blood Rev 31:37-45 (2017) DOI:10.1016/j.blre.2016.08.002 PMID:24476525 AUTHORS Tahlan A, Ahluwalia J TITLE Factor XIII: congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit. JOURNAL Arch Pathol Lab Med 138:278-81 (2014) DOI:10.5858/arpa.2012-0639-RS PMID:22477542 AUTHORS Ichinose A TITLE Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control. JOURNAL Int J Hematol 95:362-70 (2012) DOI:10.1007/s12185-012-1064-3 |