eJHaem
When Liver Disease Misleads the Diagnosis: Two Cases of TAFRO Syndrome With Porto‐Sinusoidal Vascular Disease
By Laura Dassy, Fanny Poletti, Kaoutar Hafraoui and 10 others.
Abstract
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.





