background congenital factor xiii (fxiii) deficiency is a rare severs autosomal recessive bleeding disorder. objectives the aim of the study was to determine the c559t > c fxiiia genotype frequency in patients with fxiii hemophilia who lived in sistan and balouchestan province in southeast of iran. patients and methods we determined the genotype of 180 patients with factor xiii hemophilia by te...

To understand the molecular pathology of factor XIII (FXIII) deficiency in vivo, its A subunit (FXIIIA)-knockout (KO) mice were functionally analyzed. Although homozygous FXIIIA female KO mice were capable of becoming pregnant, most of them died due to excessive vaginal bleeding during gestation. Abdominal incisions revealed that the uteri of the dead mice were filled with blood and that some e...

BACKGROUND Homozygous or double heterozygous factor XIII (FXIII) deficiency is characterized by soft tissue hematomas, intracranial and delayed spontaneous bleeding. Alterations of thromboelastography (TEG) parameters in these patients have been reported. The aim of the study was to show results of TEG, TEG Lysis (Lys 60) induced by subthreshold concentrations of streptokinase (SK), and to comp...

Acquired factor XIII (FXIII) deficiency can result in life-long bleeding tendency and be caused by enhanced consumption, impaired synthesis, or as an immune-mediated process. The latter related with solid neoplasms, through neutralizing non-neutralizing antibodies. relationship between FXIII activity non-small cell lung cancer (NSCLC) is not well established. This case report about a patient NS...

Alterations in the haemostatic system were characterized in cats with different naturally occurring liver diseases. The study looked at 44 healthy cats and 45 cats with different liver diseases confirmed histologically or cytologically (neoplasia, n=9; inflammation, n=12; hepatic lipidosis, n=13; other degenerative liver diseases, n=11). The following parameters were evaluated: platelet count; ...

FXIII, also called fibrin-stabilising factor [1] , is a transglutaminase composed of two A subunits and two B subunits circulating in plasma as a tetramer (A2B2) (Figure 1). FXIII plays an important role in clot stabilisation by cross-linking fibrin chains [2] after activation by thrombin (Figure 1). In addition, a number of other proteins are substrates for activated FXIII, such as α2-antiplas...

During the haemostatic response, the formation of a primary platelet plug limits bleeding and provides a surface for clotting factors to assemble and become activated. The initial platelet plug is stabilized by fibrin monomers, covalently cross-linked by FXIII, forming a platelets-fibrin thrombus. Defects in platelets as well as inherited deficiencies of coagulation factors including fibrinogen...

light of the results of the French study, one can only agree with the recommendation of Lepretre et al that FCA should not be used for the treatment of CLL outside of clinical trials. The question of whether alemtuzumab should no longer be used in any chemoimmu-notherapy is an entirely different issue. It should be emphasized that a recent random-ized trial showed that the second-line use of fl...