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Jul 13, 2025, 15:35

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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