Hemophilia is a hemorrhagic disorder with sex-linked inherited pattern, characterized by an inability to amplify coagulation due deficiency in factor VIII (hemophilia A or classic) IX B). Sequencing of the genes involved hemophilia has provided description and record main mutations, as well correlation various degrees severity. Hemorrhagic manifestations are related levels circulating factor, m...

background bone marrow transplantation (bmt)is nowadays used in various hematological disorders including leukemias. hemophilia a & b are sex linked bleeding disorders in which there are various genetic abnormalities in factor viii & ix genes. among various hematological disorders, bleeding disorders mainly hemophilia in now widely treated using plasma derived and recombinant factor viii & ix c...

AIM This case report presents envenoming by the Chinese pit viper Protobothrops mangshanensis (formerly Zhaoermia) and its treatment. METHODS A 38 year old snake breeder suffered two-fang bites to elbow by a Chinese pit viper Protobothrops mangshanensis resulting in local edema of the affected arm. No other signs of envenoming appeared. On the 5(th) day following the bite a hematoma developed...

von Willebrand disease is the most common inherited bleeding disorder, with mucocutaneous and menorrhagia as leading clinical manifestations. Cause of diathesis deficit or dysfunction vonWillebrand factor, plasma protein important roles in adhesion platelets to site vascular injury transport protection coagulation factor VIII. Prevalance disease, according different registries, between 1 on 100...

INTRODUCTION Idiopathic purpura fulminans is a cutaneous thrombotic disorder usually caused by autoimmune-mediated protein C or S deficiency. This disorder typically presents with purpura and petechiae that eventually slowly or rapidly coalesce into extensive, necrotic eschars on the extremities. We present the first known case of idiopathic purpura fulminans consistent with prior clinical pres...

Hemophilia is a disease of blood coagulation inherited as a sex linked recessive. The disorder is attributed to decreased blood levels of procoagulant factor VIII. However, mild forms of the disease with almost inapparent clotting defects have been reported. Patients with hemophilia often give a history of unusual bleeding associated with minor trauma. The disease may however remain undetected ...

Over the past century, the congruency between the modern description of hemophilia and a hereditary coagulation disorder described in the Talmud has been widely noted. Examination of the Talmudic text suggests that: 1. The disease described there closely resembles the modern characterization of classic hemophilia. 2. The hereditary pattern described is consistent with the distinctive inheritanc...

Background Hemostatic disorder and massive bleeding are common complications after cardiac surgery procedures, particularly in procedures requiring prolonged cardiopulmonary bypass. Conventional laboratory coagulation tests failed to predict hemostatic disorder and consequent proclivity to excessive bleeding. On the other hand there is evergrowing proportion of patients who need antiplatelet th...

von Willebrand disease (VWD) is the commonest inherited bleeding disorder. The aim of therapy for VWD is to correct the two defects of haemostasis in this disorder, impaired primary haemostasis because of defective platelet adhesion and aggregation and impaired coagulation as a result of low levels of factor VIII. The objective of this guideline is to inform individuals making choices about the...

BACKGROUND Venous thromboembolism (VTE), including both deep vein thrombosis (DVT) and pulmonary embolism (PE), is a common, lethal disorder that affects hospitalized and non-hospitalized patients. This study aimed to review the progress in the research into VTE. DATA SOURCES We reviewed the studies about VTE and verified different genetic polymoriphisms of VTE. RESULTS The pathogenesis of ...