Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resis...

The prognosis of patients with myocardial infarction (MI) and normal coronary arteries (NCA) in the presence of an inherited coagulation disorder is unknown. The purpose of this study was to compare the clinical thrombosis outcome of patients with (GpI) or without (GpII), inherited coagulation disorders, who suffered from an acute MI with NCA. Eighty two consecutive patients (mean age 49 +/- 15...

Protein S is a glycoprotein essential in the regulation of blood coagulation. Familial protein deficiency increases risk venous thromboembolism approximately 2- to 11-fold. Herein, we report this disorder six members Vietnamese family among which three had thromboembolism, and other were asymptomatic. The levels ranged from 10.1% 24%, but did not identify any PROS1 mutation. In one patient, rar...

BACKGROUND Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare condition which usually manifests as multiple hemangioma-like skin and gastrointestinal lesions. The latter often present with chronic bleeding. There is no consensus regarding the optimal management of such patients. Although rare, complications such as intestinal intussusception might occur, demanding surgical treatment. Postoperati...

Hemophilia is a hereditaty coagulation disorder which is inherited as an X-link recessive trait. Disorder happens in the intrinsic hemostasis pathway, in which there is a deficiency or defect of coagulation factor VIII (Hemophilia A) or IX (Hemophilia B). Hemophilia is comnron in male, though occurrence in female has also been reported. Female usually are carries. Immunologically, there are sev...

The case is described of a young man with probable familial antithrombin III deficiency, a disorder associated with a marked predisposition to venous thrombo-embolic events. In addition to a history of venous thrombosis and pulmonary embolism, at post-mortem this patient demonstrated widespread arterial thrombosis and atheroma. The probable association of severe arterial thrombosis and atheroma...

Introduction. Platelet dense granule disorders are a group of rare heterogeneous the blood coagulation system in which bleeding occurs due to functional and morphological platelet organelles accumulating phosphates bioactive amines. Aim — present clinical case 37-year-old patient with severe hemorrhagic syndrome. Basic information. An observation occurrence manifestations unspecified genesis is...

The development of a purpura-fulminans-like disorder which is a human equivalent of a local Shwartzman reaction in a woman with active chronic hepatitis is described. The cyclical appearance of blue-black, well circumscribed, haemorrhagic, acutely painful lesions in the buttocks, over the lateral aspects ofthe thighs, and on the arms suggested the diagnosis. Evidence of increased intravascular ...

introduction: the main inhibitors of coagulation pathway are antithrombin (at), protein c and protein s. these inhibitors are necessary to prevent thromboembolism. hereditary deficiency of inhibitors is the main cause of alteration in balance between the anti-clotting and the formation of thrombin. patients with this abnormality are susceptible to venous thromboembolism (vte). two major clinica...