Early plasmapheresis in type 2 benign recurrent intrahepatic cholestasis:A case report and review of literature  

作　　者：Lander Heyerick Annemieke Dhondt Hans Van Vlierberghe Xavier Verhelst Sarah Raevens Anja Geerts 

机构地区：[1]Department of Gastroenterology and Hepatology,Ghent University Hospital,Ghent 9000,Belgium [2]Department of Nephrology,Ghent University Hospital,Ghent 9000,Belgium

出　　处：《World Journal of Hepatology》2025年第2期279-285,共7页世界肝病学杂志(英文)

摘　　要：BACKGROUND Benign recurrent intrahepatic cholestasis(BRIC)is a rare autosomal recessive liver disease,causing episodic cholestasis with intense pruritus.This case report highlights the effectiveness of early plasmapheresis as a therapeutic option for BRIC type 2,offering rapid symptom relief and early termination of cholestatic episodes.It contributes to the limited evidence supporting plasmapheresis as a treatment for BRIC flares resistant to conventional therapies.CASE SUMMARY A 43-year-old male with BRIC type 2 presented with fatigue,jaundice,and severe pruritus,triggered by a recent mild severe acute respiratory syndrome coronavirus 2 infection.Laboratory results confirmed cholestasis with elevated bilirubin and alkaline phosphatase.First-line pharmacological treatments,including cholestyramine and rifampicin,failed.Endoscopic nasobiliary drainage was ineffective,prompting initiation of plasmapheresis.This intervention rapidly relieved pruritus,with complete biochemical normalisation after 11 sessions.Two years later,a similar episode occurred,and early reinitiation of plasmapheresis led to symptom resolution within two sessions and biochemical recovery within two weeks.The patient tolerated the procedure well,with no adverse effects observed.Follow-up showed no signs of cholestasis recurrence.CONCLUSION Plasmapheresis is a safe and effective option for therapy-refractory BRIC type 2,particularly when initiated early in cholestasis.

关 键 词：CHOLESTASIS Benign recurrent intrahepatic 2 PLASMAPHERESIS PRURITUS CHOLESTASIS Bile salt export pump Case report 

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