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We describe a case of impending acute liver failure in a patient with Sézary syndrome (SS). The three-phase computed tomography (CT) of the liver showed neither mass nor hepatomegaly. Liver biopsy confirmed infiltration with malignant CD4+ clonal T-cells. Prompt administration of combination chemotherapy, consisting of gemcitabine, dexamethasone, and cisplatin (GDP), resulted in full recovery of liver function. To the best of our knowledge, this is the first report of liver failure from SS. Commercial next-generation sequencing panel identified 11 clinically relevant mutations. Interestingly, the identified ARID2 mutation, frequently observed in hepatocellular carcinoma, rarely occurs in hematologic malignancies. Further studies are necessary to elucidate the role of ARID2 mutations in the biological behavior of Sezary cells, such as a propensity to infiltrate liver parenchyma.

Hepatic Infiltration with Malignant T-cells Manifesting as Impending Acute Liver failure in Mycosis Fungoides with Large Cell Transformation