Association of genotypes of thrombin-activatable fibrinolysis inhibitors with thrombotic microangiopathies--a pilot study.

Background: Thrombotic microangiopathies are characterized by microvascular thrombosis, consequently leading to microangiopathic haemolytic anaemia, thrombocytopenia and organ dysfunction. Although recent research has elucidated the pathogenesis of these rare thrombotic disorders to some extent, the determinants contributing to the manifestation remain rather unclear in the majority of affected patients.

Method: In the present pilot study, we used a case-control design, enrolling 40 patients [mean age (+/-SD) 35+/-11 years] with a history of thrombotic microangiopathy and 689 control subjects to evaluate the association of gene polymorphisms of the thrombin-activatable fibrinolysis inhibitor (TAFI) with the manifestation of these rare thrombotic disorders.

Amyloidosis and bleeding: pathophysiology, diagnosis, and therapy.

Amyloid diseases can be associated with potentially life-threatening hemorrhage. Pathogenetic factors contributing to the abnormal bleeding tendency in this setting are heterogeneous and depend on the type of amyloidosis and pattern of organ involvement. In patients with light-chain (AL) amyloidosis, acquired hemostatic abnormalities, including coagulation factor deficiencies, hyperfibrinolysis, and platelet dysfunction, can be regarded as the most important pathogenetic factors.