We report a case of acute lymphoblastic leukemia (ALL) presenting as severe jaundice. The patient, a 59-year-old man, was found to have abnormal liver function, including an elevated total bilirubin level (13.5 mg/d
l) with hepatosplenomegaly, but no detectable lymphadenopathy. A liver biopsy and bone marrow examination revealed a lymphoid neoplasm. Pathologic features included invasion of an abnormal clone into the sinusoidal region of the liver, diffuse bone marrow involvement (41.6% of all nucleated cells) and splenomegaly. Small numbers of malignant cells were also detected in the peripheral blood. B-cell markers, such as terminal deoxynucleotidyl transferase (TdT), CD10, CD19, CD20 and HLA-DR were positive, and CD2, CD3, CD4, CD5, CD7, CD8, κ, λ, cytoplasmic μ and myeloperoxidase were negative. Cytogenetic analysis detected hyperdiploidy. In this case, a dose-attenuated CHOP regimen attained complete remission. To date, preferential infiltration to liver sinusoids has been noted in hepatosplenic γ/δ T-cell lymphoma, other NK/T-cell malignancies, and some cases of hairy cell leukemia. Severe jaundice due to preferential infiltration of leukemic cells into liver sinusoids is rather uncommon as a presenting feature of ALL.
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