introduction: the prevalence of rare bleeding disorders, including combined factor v+viii deficiency are higher in iran than in developed countries. there are only a few reports which have been written concerning kidney transplantation in the patients suffering from these disorders. case report: a 22-year old girl, with a known case of combined factor v+viii deficiency, a history of bladder sto...

background: the most common polymorphisms identified in the methylenetetrahydrofolate reductase (mthfr) gene, c677t and a1298c lead to defective activity of this enzyme and increase the risk of venous and arterial thrombosis. there are limited investigations regarding the effects of thrombogenic polymorphisms on the clinical phenotypes of rare hereditary hemorrhagic disorders like glanzmann's t...

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...

Objective: The incidence and range of rare hereditary bleeding diseases, as well the severity deficiency its many clinical presentations, were subjects our study. Study Design: Cross-sectional study Place Duration: Department Pathology, Northwest School Medicine Peshawar in duration from July, 2022 to December, 2022. Methods: Total 850 cases coagulation disorders presented this A thorough demog...

inhibitor development is a lifelong challenge for patients with bleeding disorders who received replacement therapy. most commonly, inhibitor formation was observed in hemophilia a patients but patients with rare bleeding disorders (rbd) especially patients with deficiency of factor xiii (fxiii) and factor v (fv) can develop an inhibitor against exogenous factors. several factors considered as ...

rare bleeding disorders (rbds) are a heterogeneous group of disorders including different types of coagulation factor deficiencies. the disorders are inherited in an autosomal recessive manner with different frequencies varying from 1:500000 to 1:2000000. patients affected with rbds are presented with a wide spectrum of clinical manifestations ranging from mild to life threatening bleeding diat...