Learn About PLGD
Plasminogen Deficiency (PLGD) is a rare condition that occurs when plasminogen (PLG) – a protein made in the liver – is reduced or almost absent.¹ Patients with PLGD develop coarse, woody-like lesions on the thin layers (mucous membranes) of the skin and organs throughout the body. Some of the more common areas where these lesions can appear are eyes, ears, gums, respiratory tract and urinary tract.²,³ These lesions may cause pain or discomfort and can affect how a person’s organs work. Emergencies, such as a collapsed lung, kidney failure, and blindness may occur if these lesions are left untreated. In some cases, complications can be fatal.³
Patients with congenital PLGD may have abnormally low, or almost no, PLG present in the blood. Others may produce some PLG, but a significant portion of it is not active or not performing normally. People with moderate levels of PLG (levels are slightly below normal) may not experience symptoms at all.²
PLGD Therapy and Clinical Trials
A variety of treatments have been tried in the management of PLGD (cyclosporine A, heparin, anti-inflammatories and steroids). These have offered very little benefit or clinical improvement. The administration of PLG appears to provide the most relief and greatest benefit.
What is PLG’s role in the body?
PLG is a protein that is made by the liver. It circulates in the blood. PLG activators convert the protein into plasmin. Plasmin breaks down fibrin, the main protein that clots blood, so normal tissue can grow back.⁸
When PLG is reduced, there is less plasmin to break down the fibrin, leading to an abnormally high build-up of fibrin. These fibrin deposits cause swelling in the tissue, resulting in lesions that are characteristic of congenital PLGD.⁸
PLG contributes to normal functioning in the body, allowing new blood vessels to form. It also aids in the development of fertilized eggs during a woman’s pregnancy.⁹
PLGD is a rare disorder that affects a small population – just one or two people out of a million. It occurs in patients whose bodies make little or no Plasminogen.¹
Rate of PLGD occurence in different parts of the world (estimated)¹
- Minnesota: 0.35%
- Southern Germany: 0.13%
- Scotland: 0.26%
- Japan: 0.42%
1. Tefs, K, Gueorguieva M, Klammt J, et al. Molecular and clinical spectrum of type I plasminogen deficiency: a series of 50 patients. Blood. 200;108(9);30-21-3026.
2. Shoseyov D. Congenital plasminogen deficiency with respiratory complication. Powerpoint presentation at Hadassah Medical Center Jerusalem: Jerusalem, Israel.
3. Mehta R, Shapiro AD. Plasminogen activator inhibitor type I deficiency. Haemophilia. 2008;14(6);1255-1260.
4. Heidemann DG, Williams GA, Hartzer M, Ohanian A, Citron ME. Treatment of ligneous conjunctivitis with topical plasmin and topical plasminogen. Cornea.2003;22(8):760-762.
5. Watts P, Suresh P, Mazer E, et al. Effective treatment of ligneous conjunctivitis with topical plasminogen. AM J Ophtalmol. 2002;133(4):451-455.
6. Pergantou H, Likkaki D, Fotopoulou M, Katsarou O, Xafaki P, Platokouki H. Management of ligneous conjunctivitis in a child with plasminogen deficiency. Eur J Pediatr. 2011,170(10):1333-1336.
7. Tabbara KF. Prevention of ligneous conjunctivitis by topical and subconjunctival fresh frozen plasma. Am J Ophtalmol. 2004;138(2):299-300.
8. National Institutes of Health, US National Library of Medicine, 2012. Genetics Home Reference: Congenital plasminogen deficiency. https://ghr.nlm.nih.gov/condition/congenital-plasminogen-deficiency. Accessed May 14, 2016.
9. Castellino FJ, Ploplis VA. Structure and function of the plasminogen/plasmin system. Thromb Haemost. 2005;93(4):647-654.
Join the Plasminogen Deficiency Community
Are you concerned about this rare disease?
We’re here to help.
Subscribe to the community to receive periodic information on plasminogen deficiency.
You are not alone!