EJHaem · 2026
When Liver Disease Misleads the Diagnosis: Two Cases of TAFRO Syndrome With Porto-Sinusoidal Vascular Disease.
СтатьяНазвание и аннотация: Перевод пока не подготовлен
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Dassy Laura, Poletti Fanny, Hafraoui Kaoutar, Lejeune Marie, Somja Joan, Collins Patrick, Guiot Julien, Theate Ivan, Caers Jo, Delwaide Jean, Marot Astrid, Putzeys Virginie, Vertenoeil Gaëlle
Аннотация
BACKGROUND: TAFRO syndrome is a rare inflammatory disorder whose hepatic manifestations may mimic decompensated cirrhosis.
CASES: We report two women with TAFRO syndrome associated with portal-sinusoidal vascular disorder (PSVD), a rare cause of non-cirrhotic portal hypertension. In both cases, liver biopsy excluded cirrhosis and confirmed PSVD.
RESULTS: Clinical outcomes were markedly different. One patient achieved sustained disease control with anti-IL-6 therapy and remained stable for more than 6 years, whereas the other developed rapidly progressive and fatal disease.
CONCLUSION: PSVD may represent an underrecognized hepatic manifestation of TAFRO syndrome. Recognizing this association may facilitate earlier diagnosis, avoid misclassification as cirrhosis, and support timely therapeutic management.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.
CASES: We report two women with TAFRO syndrome associated with portal-sinusoidal vascular disorder (PSVD), a rare cause of non-cirrhotic portal hypertension. In both cases, liver biopsy excluded cirrhosis and confirmed PSVD.
RESULTS: Clinical outcomes were markedly different. One patient achieved sustained disease control with anti-IL-6 therapy and remained stable for more than 6 years, whereas the other developed rapidly progressive and fatal disease.
CONCLUSION: PSVD may represent an underrecognized hepatic manifestation of TAFRO syndrome. Recognizing this association may facilitate earlier diagnosis, avoid misclassification as cirrhosis, and support timely therapeutic management.
TRIAL REGISTRATION: The authors have confirmed clinical trial registration is not needed for this submission.