World’s First Liver Transplant for Plasminogen Deficiency Performed at KFSHRC
Dr. Chokri Ben Lamine, hematologist at King Faisal Specialist Hospital & Research Centre (KFSHRC), recently shared an exciting update on X:
“Historic First: Dr. Hazzaa Alzahrani (Adult Hematologist, KFSHRC) with Dr. Broering (Liver Transplant Team) performed the world’s first liver transplant for plasminogen deficiency, restoring plasminogen production and fibrinolysis successfully.Plasminogen Deficiency: Key Review
Definition: Rare AR (PLG mut) → ↓plasminogen → ↓fibrinolysis → fibrin-rich pseudomembranes (ligneous conjunctivitis, gingivitis, RT/GU obstruction).
Types:
Type I: ↓ antigen & activity
•Type II: nl antigen, ↓ activity
Clinical: Ligneous conjunctivitis (80%), gingivitis, RT/GU obstruction, hydrocephalus.
Diagnosis:
•↓ Plasminogen activity (functional assay)
•↓ Antigen (ELISA)
•Genetic testing
Management:
Supportive:
•Lubricants, surgical removal + fibrinolytics
Replacement Therapy:
•FFP 10–15 mL/kg q2–3w
•RYPLAZIM: 6.6 mg/kg q2d x6, then weekly FDA
Topical fibrinolytics: tPA, urokinase
Adjuncts: Avoid trauma, treat infections promptly
Experimental: Gene therapy & recombinant plasminogen trials
Complications: Airway obstruction, vision loss, renal involvement (rare)
Pearls:
•Consider in recurrent ligneous conjunctivitis
•Monitor plasminogen levels
•Early replacement prevents complications
References:
•FDA RYPLAZIM
•Schuster V et al. Haemophilia. 2000;6(3):162-172 PubMed
•Tefs K et al. Blood. 2006;107(12):4823-4830 DOI.”
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