A specific assay for alpha 2-antiplasmin deficiency is performed when a patient has a bleeding diathesis that cannot be identified by the usual screening assays. Both alpha 2-antiplasmin antigen and activity levels can be measured.
Two types of alpha 2-antiplasmin deficiency exist:
Homozygous patients usually have undetectable levels while heterozygous patients have levels 40% – 60% of normal. The normal plasma concentration is 0.7 mg/L.
Imaging techniques are used for acute events, as applicable. The utilization of imaging techniques depends on the site of bleeding and the necessity of imaging. The unique intramedullary hematomas are visualized by magnetic resonance imaging as homogenous hyperintense signals in the medulla and hypointense signals surrounding the lesion.
Rare Coagulation Disorders Resource Room, an extension of the RBDD Registry (eu.rbdd.org), was developed in collaboration with the Indiana Hemophilia & Thrombosis Center (www.innovativehematology.org).
The Resource Room serves as a platform to promote international collaboration to increase knowledge of these very rare disorders and to improve the care of affected individuals. The international RBDD mission, under the leadership of Dr. Peyvandi, will continue to be expanded over time.
The Resource Room thanks all contributing authors of the website articles that have made this valuable information available to the global community of both individuals affected with rare coagulation disorders and their healthcare providers.
