Acquired plasma factor XIII deficiencies.
Coagulation factor XIII (FXIII) is of paramount importance in the process of fibrin stabilization, which is the final step of the coagulation cascade. The clinical significance of defective fibrin stabilization is highlighted by the severe hemorrhagic manifestations of congenital FXIII deficiency. In this paper we review the pathophysiology, clinical presentation and therapy of acquired plasma FXIII deficiencies, caused by specific inhibitors or associated with other clinical conditions. For acquired severe FXIII deficiency caused by factor-specific inhibitors, the need for prompt diagnosis and treatment is emphasized by the high hemorrhagic risk and mortality. For moderate reduction of FXIII secondary to other conditions, we discuss the relative importance of FXIII reduction in the development of clinical symptoms and the role of substitution treatment.
Tosetto
A
A
Department of Hematology, San Bortolo Hospital, Vicenza, Italy.
Castaman
G
G
Rodeghiero
F
F
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000595:Amino Acid Sequence; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000276:immunology; D002648:Child; D003429:Cross Reactions; D004789:Enzyme Activation; D005176:Factor XIII / Q000037:antagonists & inhibitors / Q000276:immunology / Q000502:physiology* / Q000627:therapeutic use; D005177:Factor XIII Deficiency / Q000139:chemically induced / Q000145:classification / Q000276:immunology / Q000628:therapy; D005260:Female; D005337:Fibrin / Q000378:metabolism; D006474:Hemorrhagic Disorders / Q000209:etiology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D015212:Inflammatory Bowel Diseases / Q000150:complications; D007538:Isoniazid / Q000009:adverse effects / Q000276:immunology; D007938:Leukemia / Q000150:complications; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D013917:Thrombin / Q000378:metabolism
D001323:Autoantibodies; D007074:Immunoglobulin G; D005337:Fibrin; D005176:Factor XIII; D013917:Thrombin; D007538:Isoniazid