Successful competition of activated factor VII (FVIIa) with zymogen factor VII (FVII) for tissue factor (TF) and loading of the platelet surface with FVIIa are plausible driving forces behind the pharmacological effect of recombinant FVIIa (rFVIIa) in hemophilia patients. Thrombin generation measurements in platelet-rich hemophilia A plasma revealed competition for TF, which potentially could r...

T-lymphocyte populations in 12 apparently healthy heterosexual adult patients with hemophilia have been examined to ascertain if these patients have abnormalities in their lymphocyte subpopulations similar to those in homosexual men, Haitian refugees, and narcotic addicts. A striking reduction in the helper to suppressor cell ratio ([0.86 +/- 0.14]:1) was found in 9 of the 12 patients. These ab...

C LASSIC HEMOPHILIA ( Hemophilia A) is a hereditary hemorrhagic disorder characterized by low to absent levels of anti-hemophiliac globulin ( AHF, Factor VIII ) . The bleeding time and other measurements of platelet function are usually described as normal. We have recently studied a patient with severe AHF-deficient hemophilia who has repeatedly developed a prolonged bleeding time and other si...

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 ...

From its original envisioned use in patients with hemophilia and inhibitors, recombinant factor VIIa has been increasingly used in a variety of non-hemophilia bleeding/hemorrhagic situations with great efficacy. Most of the reported work has been in adult patients. This paper sets out to review its use in the pediatric non-hemophilia patients and the varied conditions it has been tried and used...

The development of an antibody in people with hemophilia to products used in the treatment and prevention of bleeding, also referred to as an inhibitor, is the most serious complication of hemophilia care today. CDC, together with healthcare providers, consumer organizations, hemophilia organizations, and federal partners, has developed a public health agenda to prevent the development of inhib...

To the Editor, I would like to highlight 3 of our patients, a 14-year-old boy and females of 4 and 41 years old [1,2] with acquired hemophilia B seen at İhsan Doğramacı Children’s Hospital (previously Hacettepe) between 1963 and 1973 among 343 patients with hemophilia [3] on account of the case of acquired hemophilia A in a 78-year-old man who was successfully treated with a combined immunosupp...

Hemophilia A is a worldwide disorder of coagulation system. It is a male disorder, yet females with hemophilia are rarely seen in communities with high rate of consanguineous marriages. The abnormalities in factor VIII gene transfer as an X-linked pattern in the family, affects as many as one-third of patients who had no family history of abnormality and thus the occurrence of a sporadic mutati...

Animal models of the bleeding disorder, hemophilia A, have been an integral component of the biopharmaceutical development process and have facilitated the development of recombinant coagulation factor VIII (fVIII) products capable of restoring median survival of persons with hemophilia A to that of the general population. However, there remain several limitations to recombinant fVIII as a biot...

UNLABELLED ESSENTIALS: Anti-factor VIII (FVIII) inhibitory antibody formation is a severe complication in hemophilia A therapy. We genetically engineered and characterized a mouse model with complete deletion of the F8 coding region. F8(TKO) mice exhibit severe hemophilia, express no detectable F8 mRNA, and produce FVIII inhibitors. The defined background and lack of FVIII in F8(TKO) mice will ...