Autoimmune acquired factor XIII (F13) deficiency

Definition:

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.

References:

[1]. Akitada Ichinose, et al. Autoimmune acquired factor XIII deficiency due to anti-factor XIII/13 antibodies: A summary of 93 patients. Blood Rev. 2017 Jan;31(1):37-45. [Content Brief]

[2]. Akitada Ichinose, et al. Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control. Int J Hematol. 2012 Apr;95(4):362-70. [Content Brief]

[3]. Anita Tahlan, et al. Factor XIII: congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit. Arch Pathol Lab Med. 2014 Feb;138(2):278-81. [Content Brief]

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