What are the best treatments for Plasminogen Deficiency (PLGD)?
See the best treatments for Plasminogen Deficiency (PLGD) here
Treatments for Plasminogen Deficiency (PLGD)
Plasminogen Deficiency (PLGD) is a rare genetic disorder characterized by the insufficient production or dysfunction of plasminogen, a protein involved in the breakdown of blood clots. This condition can lead to the formation of abnormal fibrin deposits, which can cause various health complications. While there is no cure for PLGD, several treatment options can help manage the symptoms and improve the quality of life for affected individuals.
Plasminogen Replacement Therapy
Plasminogen replacement therapy is the primary treatment for PLGD. It involves administering purified plasminogen derived from human plasma to supplement the deficient levels in the patient's body. This therapy aims to restore the normal functioning of plasminogen and prevent the formation of abnormal fibrin deposits. Plasminogen replacement therapy can be administered intravenously, and the dosage and frequency depend on the severity of the condition and individual patient needs.
Topical Plasminogen Therapy
In addition to intravenous administration, topical plasminogen therapy can be used for specific manifestations of PLGD, such as ligneous conjunctivitis. This approach involves applying plasminogen eye drops or ointments directly to the affected areas, promoting the dissolution of fibrin deposits and reducing inflammation. Topical plasminogen therapy can help manage ocular symptoms and prevent complications associated with ligneous conjunctivitis.
Surgical Interventions
In some cases, surgical interventions may be necessary to manage PLGD-related complications. For instance, if abnormal fibrin deposits obstruct blood vessels or cause organ damage, surgical removal or bypass procedures may be required. These interventions aim to restore blood flow, alleviate symptoms, and prevent further complications. Surgical options should be considered on a case-by-case basis, and the decision should involve a multidisciplinary team of healthcare professionals.
Supportive Care
Supportive care plays a crucial role in managing PLGD and improving the overall well-being of affected individuals. This includes regular monitoring of plasminogen levels, fibrin deposition, and associated symptoms. Close collaboration with healthcare professionals, such as hematologists, ophthalmologists, and genetic counselors, is essential to ensure appropriate management and early intervention. Additionally, symptomatic treatment can be provided to address specific manifestations of PLGD, such as anti-inflammatory medications for joint pain or physical therapy for musculoskeletal complications.
Genetic Counseling
Genetic counseling is an integral part of PLGD management, especially for individuals and families affected by this rare genetic disorder. Genetic counselors can provide information about the inheritance pattern, genetic testing options, and the risk of passing the condition to future generations. They can also offer emotional support, help individuals make informed decisions, and connect them with relevant support groups or resources.
In conclusion, while there is no cure for Plasminogen Deficiency (PLGD), various treatment approaches can help manage the symptoms and improve the quality of life for affected individuals. Plasminogen replacement therapy, topical plasminogen therapy, surgical interventions, supportive care, and genetic counseling are essential components of a comprehensive treatment plan. Early diagnosis, close monitoring, and a multidisciplinary approach involving healthcare professionals are crucial for optimal management of PLGD.
PLASMINOGEN CONCENTRATES
Using fresh frozen plasma as a source of plasminogen, PLG concentrates given locally (specific to one area) and systemically (circulating throughout the body) have been shown to provide some symptom relief and may prevent lesions from reforming.
Locally administered fresh frozen plasma (which contains a low concentration of plasminogen) and other PLG-containing eye drops (generated from fresh frozen plasma) have shown some effectiveness in treating eye lesions associated with ligneous conjunctivitis. These drops require administration 3 to 4 times a day and are not widely commercially available. .
Purified Plasminogen Intravenous Therapy
Clinical trial results involving systemic treatment with purified IV plasminogen for patients with congenital plasminogen deficiency is currently under review by the FDA (2017). Results reported to date indicate that purified IV plasminogen therapy is effective at reducing and resolving both visible lesions, such as ligneous conjunctivitis and systemic lesions, such as those that arise in the respiratory tract ureter, uterus or on other mucosal surfaces. Patients in the trial had a 100% response rate and 90.9% of visible lesions were completely resolved after 12 weeks of therapy. Plasminogen therapy effectively prevented new lesions from forming during this 12-week interim analysis. Final results from the long-term, 48-week trial are not yet published. (C. Nakar, A. Shapiro et al., ISTH, 2017).
Posted Aug 9, 2017 by jodoinjulie 2000
Posted May 9, 2020 by Maria Kato 200
