ristocetin and Factor-XIII-Deficiency

von Willebrand disease (VWD) is the most common inherited bleeding disorder, but variable severity and several classification types mean that diagnosis is often not straightforward. In many countries, the assays are not readily available and/or are not well standardized. The latest methods and the basis of VWD are discussed here, together with information from the international quality assessment programme (IEQAS). Factor XIII deficiency is a rare, but important bleeding disorder, which may be missed or diagnosed late. A discussion and update on this diagnosis is considered in the final section of our review.

Topics: Clinical Laboratory Techniques; Collagen; Factor XIII Deficiency; Hemagglutinins; Hemophilia A; Humans; Platelet Aggregation; Quality Control; Ristocetin; von Willebrand Diseases; von Willebrand Factor

Topics: Adenosine Diphosphate; Child; Child, Preschool; Collagen; Epinephrine; Factor XIII Deficiency; Female; Humans; Male; Platelet Aggregation; Ristocetin