ArticleEJHaem2026
When Liver Disease Misleads the Diagnosis: Two Cases of TAFRO Syndrome With Porto-Sinusoidal Vascular Disease.
Article in EJHaem, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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13 authors.
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Abstract
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.
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