deamino-arginine-vasopressin and Virus-Diseases

Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a defect in the multimeric glycoprotein called von Willebrand factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing to low plasma levels of factor VIII. There are 2 main options available for the treatment of spontaneous bleeding episodes and for bleeding prophylaxis: desmopressin and transfusional therapy with plasma products. Desmopressin is the treatment of choice for most patients with type 1 vWD, who account for approximately 70% to 80% of cases. This nontransfusional hemostatic agent raises endogenous factor VIII and von Willebrand factor 3 to 5 times and thereby corrects both the intrinsic coagulation and the primary hemostasis defects. In patients with the more severe type 3 and in most patients with type 2 disease, desmopressin is ineffective or is contraindicated and it is usually necessary to resort to plasma concentrates containing both factor VIII and von Willebrand factor. Concentrates treated with virucidal methods should be preferred to cryoprecipitate because they are equally effective and are perceived as safer. (Blood. 2001;97:1915-1919)

Topics: Blood Loss, Surgical; Combined Modality Therapy; Contraindications; Deamino Arginine Vasopressin; Endothelium, Vascular; Factor VIII; Female; Hemorrhage; Humans; Isoantibodies; Male; Myocardial Infarction; Postoperative Hemorrhage; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Receptors, Vasopressin; Safety; Stroke; Thrombocytopenia; Transfusion Reaction; Virus Diseases; von Willebrand Diseases; von Willebrand Factor

Over the past few years considerable progress has been made in elucidating the molecular genetics of hemophilia A, in carrier detection and prenatal diagnosis, and in the production of safer clotting factor concentrates. Recombinant FVIII, shown to be safe and effective in ongoing prelicensure clinical trials that began in the spring of 1987, should soon be licensed and commercially available. There is now considerable interest in beginning prophylactic therapy regimens at 1 or 2 years of age, in an attempt to prevent chronic joint disease and other complications of serious bleeding episodes. The possibility of gene insertion therapy for persons with hemophilia now seems to be a realistic one--perhaps achievable in the 1990s. Although many problems remain--major problems resulting from HIV, HCV, and HBV infections; how to deal with existing musculoskeletal problems; how to pay for the higher-priced new technologies; high titer inhibitors; just to name a few--the many recent scientific advances and their clinical applications make this an exciting time. This is truly, as indicated in the title of the proceedings of the XIX Congress of the World Federation of Hemophilia, a new decade of hopes and challenges.

Topics: Child, Preschool; Deamino Arginine Vasopressin; Dental Care; Factor VIII; Fetal Diseases; Hemarthrosis; Hemophilia A; Hemorrhage; Humans; Infant; Infant, Newborn; Prenatal Diagnosis; Prevalence; Recombinant Proteins; Virus Diseases

In von Willebrand disease, the goal of treatment is to correct the two laboratory hallmarks of abnormal hemostasis, i.e. the deficiency of factor VIII (FVIII) and the prolonged bleeding time (BT). Since desmopressin (DDAVP) is able to achieve both these goals in the majority of patients, it is the treatment of choice. Some patients, however, are not responsive or become refractory to DDAVP. In these, blood products maintain an important therapeutic role, and there is a need to assess the efficacy of the recently available virus-inactivated plasma concentrates, which contain both FVIII and von Willebrand factor and carry a low risk of transmitting blood-borne viruses. Our survey of the data reported in the literature indicates that all available concentrates are similarly effective in attaining high and sustained levels of FVIII after infusion. Although they often shorten or normalize the prolonged BT, that effect is less uniform. Since concentrates appear efficacious in the majority of clinical situations that require the use of blood products, they should be preferred, because of their greater safety, to cryoprecipitate produced by blood banks, which cannot be virus inactivated.

Topics: Bleeding Time; Blood Component Transfusion; Combined Modality Therapy; Deamino Arginine Vasopressin; Detergents; Freeze Drying; Hot Temperature; Humans; Plasma; Prevalence; Safety; Solvents; Virus Diseases; Viruses; von Willebrand Diseases; von Willebrand Factor

Topics: Blood; Deamino Arginine Vasopressin; Factor VIII; Hemophilia A; Hepatitis B Vaccines; Humans; Immunologic Deficiency Syndromes; Isoantibodies; Safety; Virus Diseases

Haemophilia is a rare and complex disorder and its successful management will depend upon the establishment of a network of 'comprehensive care' including the services of haematologists, orthopaedic surgeons, rheumatologists, dental surgeons, physiotherapists, specialised nurses and counsellors. One of the major lessons to be learned from the HIV epidemic in haemophilia is that it is critical to strive to obtain the safest and purest forms of blood products for these patients. The advent of clinically available recombinant factor VIII is expected soon; in the meantime there is a move towards treating all patients with high purity products.

Topics: Cerebral Hemorrhage; Deamino Arginine Vasopressin; Factor IX; Factor VIII; Genetic Counseling; Hemarthrosis; Hemophilia A; Hemophilia B; Humans; Male; Patient Care Team; Prevalence; Transfusion Reaction; Virus Diseases; von Willebrand Diseases