DISEASE: Autoimmune acquired factor XIII (F13) deficiency
Entry
H01759 Disease
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
Brite
Human diseases in ICD-11 classification [BR:br08403]
03 Diseases of the blood or blood-forming organs
Coagulation defects, purpura or other haemorrhagic or related conditions
Coagulation defects
Haemorrhagic diseases due to acquired coagulation factor defects
3B21 Haemorrhagic disorder due to circulating anticoagulants and coagulation factors
H01759 Autoimmune acquired factor XIII (F13) deficiency
