Hemophilia is a rare disorder that is complex to diagnose and to manage. These evidence-based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion-transmitted infections. By compiling these guidelines, the World Federation of Hemophilia aims to assist healthcare providers seeking to initiate and/or maintain hemophilia care programs, encourage practice harmonization around the world and, where recommendations lack adequate evidence, stimulate appropriate studies.

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Guidelines for the management of hemophilia.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/jth.12672

Definitions in hemophilia: communication from the SSC of the ISTH.

Bleeding after cardiopulmonary bypass remains a cause for concern, requiring reexploration of the chest in approximately 3 percent of patients who have had operations on the heart. We examined the possibility that this problem might be alleviated by desmopressin acetate (DDAVP), which increases the plasma level of von Willebrand factor and improves hemostasis in mild hemophilia and other conditions associated with defective platelet function. In a double-blind, prospective, randomized trial, we studied the effect of intraoperative desmopressin acetate in 70 patients undergoing various cardiac operations requiring cardiopulmonary bypass. Patients undergoing uncomplicated primary coronary-artery bypass grafting were not included. The drug significantly reduced mean operative and early postoperative blood loss (1317±486 ml in the treated group vs. 2210±1415 ml in the placebo group); of the 14 patients whose 24-hour blood loss exceeded 2000 ml, 11 had received the placebo. Plasma levels of von Willeb...

https://www.nejm.org/doi/pdf/10.1056/NEJM198605293142202?listPDF=true

Treatment with desmopressin acetate to reduce blood loss after cardiac surgery. A double-blind randomized trial.

Desmopressin (DDAVP) in the Treatment of Bleeding Disorders: The First 20 Years

The most common medical cause of major blood loss is surgery, particularly cardiovascular procedures, liver transplantation and hepatic resection, and major orthopedic procedures. Excessive blood loss may also occur for other reasons, such as trauma. This article reviews the benefits of hemostatic drugs and considers the associated risk of adverse events, particularly thrombotic complications.

Prevention and treatment of major blood loss.

https://www.onlinelibrary.wiley.com/doi/pdf/10.1111/j.1538-7836.2011.04214.x

A randomized clinical trial of prophylaxis in children with hemophilia A (the ESPRIT Study)

DDAVP enhances platelet adherence and platelet aggregate growth on human artery subendothelium.

In 1977 WHO published in the Bulletin a Memorandum on Methods for the Detection of Haemophilia Carriers This was produced following a WHO/WFH (World Federation of Haemophilia) Meeting of Investigators in Geneva in November 1976, and has served as a valuable reference article on the genetics of haemophilia The analyses discussed were based on phenotypic assessment, which, at that time, was the only procedure available The molecular biology revolution in genetics during the 1980s made enormous contributions to our understanding of the molecular basis of the haemophilias and now permits precise carrier detection and prenatal diagnosis WHO and WFH held a joint meeting on this subject in February 1992 in Geneva This article is the result of these discussions

P M Mannucci

Papers

A randomized clinical trial of prophylaxis in children with hemophilia A (the ESPRIT Study)

DDAVP enhances platelet adherence and platelet aggregate growth on human artery subendothelium.

Haemophilia: strategies for carrier detection and prenatal diagnosis.

Carrier detection in hemophilia A: a cooperative international study. I. The carrier phenotype

Carrier detection in hemophilia A: a cooperative international study. II. The efficacy of a universal discriminant.