BACKGROUND Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened f...

Two hemophilic beagles were given normal sibling. During observation 1200 A whole-body irradiation followed periods of 7 and 24 mo, there was no by a successful marrow graft from a evidence of factor VIII synthesis. ORTHOTOPIC TRANSPLANTATION of a normal liver into a hemophilic dog results in complete correction of the deficiency of factor VIII.”3 However, there are significant extrahepatic sou...

Von Willebrand disease is a common autosomal inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor, a multi-adhesive protein that binds platelets to exposed subendothelium and carries factor VIII in circulation. As a result of von Willebrand factor deficiency or abnormality, levels of factor VIII, the protein deficient in hemophilia A, may be variabl...

H EMOSTATIC DISORDERS with a varying incidence of bleeding, are common in the dysproteinemias, and are characteristic of the macroglobulinemia of \\Taldenstr#{246}m These abnormalities are most frequently attributed to abnormal platelet function,1 and depression of specific clotting factors appears to be a rare occurrence. Nilehn2 includes four instances of antihemophilic globulin ( factor VIII...

TO THE EDITOR: Combined factor V and VIII deficiency, a rare autosomal recessive coagulopathy, was first defined by Oeri et al. in 1954 [1]. Although it is seen worldwide, Mediterranean countries have higher prevalence rates [2]. The presentation varies from mild to serious bleeding symptoms such as easy bruising, menorrhagia, epistaxis, gingival bleeding, intramuscular bleeding, and post-opera...

We report a study on the importance of factor IX activation in thromboplastin-dependent coagulation in plasma. Diluted, CaCl2-containing thromboplastin solutions at constant phospholipid concentration were used to trigger the coagulation in plasma from patients with congenital factor IX and factor VIII deficiency in the presence and absence of added factors IX and VIII, and the generation of th...

Individuals with hemophilia are deficient in one of the clotting factor proteins that are vital in the formation of a clot. Classic hemophilia or hemophilia A is a deficiency of factor VIII, while Christmas Disease or Hemophilia B is a deficiency of factor IX. The deficiency of one of these proteins comes about because of a mutation on the X chromosome. Patients with both types of hemophilia ar...

Postpartum-acquired hemophilia A is a rare and potentially severe complication of pregnancy caused by an autoantibody against factor VIII [1]. Acquired factor VIII deficiency is associated with autoimmune conditions, neoplastic diseases, drug hypersensitivity, and pregnancy. A retrospective study analyzed 51 published cases of postpartum factor VIII inhibitors, with regard to the outcome accord...