fibrin and Lymphoproliferative-Disorders

Coagulation factor XIII (FXIII) is a protein that promotes fibrin stabilization by forming multiple covalent cross-links between fibrin monomers. Beside congenital FXIII deficiency, due to FXIII gene mutations, severe acquired FXIII deficiency has been described in association with autoantibodies against coagulation FXIII. These inhibitors, which occurs very rarely but may cause life-threatening bleeding complications, may arise spontaneously or in association with autoimmune and lymphoproliferative disorders or medications. The management of patients with acquired FXIII inhibitors is very demanding and treatment regimens must be focused on eradication of the inhibitor and to increase the plasma FXIII levels. In this systematic review, we analyse all the published case-reports on anti-FXIII autoantibodies focusing on the clinical features and treatment modalities of this acquired hemorrhagic condition.

Topics: Autoantibodies; Autoimmune Diseases; Blood Coagulation Factor Inhibitors; Factor XIII; Factor XIII Deficiency; Fibrin; Humans; Lymphoproliferative Disorders

Topics: Adult; Aged; Aged, 80 and over; Autoantibodies; Autoantigens; Autoimmune Diseases; Biopolymers; Blood Coagulation Disorders; Blood Coagulation Factors; Female; Fibrin; Hemorrhagic Disorders; Humans; Lymphoproliferative Disorders; Male; Middle Aged; Paraproteins; Thrombophilia

Topics: Epstein-Barr Virus Infections; Fibrin; Hematoma; Herpesvirus 4, Human; Humans; Lymphoma, Large B-Cell, Diffuse; Lymphoproliferative Disorders; T-Lymphocytes