In patients with lesions suspected to be ligneous, a plasminogen antigen and activity level should be obtained. As the majority of clinical lesions have been associated with type I deficiency, it is important to evaluate both assays to demonstrate a decreased plasminogen activity with concordant decrease in protein level. Plasminogen activity is measured using a chromogenic assay with typical normal range of 70-130%; while antigenic testing is commonly performed via immunologic assays with a reported normal range of 6-25 mg/dL.1 By the age of 1 year, plasminogen levels stabilize and do not subsequently vary with age.47
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.
