PRIMARY SINUSOIDAL LYMPHOMA OF THE LIVER REVEALED BY AUTOIMMUNE HEMOLYTIC-ANEMIA

Primary liver lymphomas usually present with the clinical picture of a liver tumor, and are characterized by a predominantly portal invasion by lymphoid cells of the B-cell phenotype. We report a case of primar y sinusoidal lymphoma of the liver, in a 36 year-old male patient, rev ealed by homogeneous hepatosplenomegaly and infiltration of liver sinu soids by morphologically normal lymphocytes, without destruction of th e parenchyma. Immunohistochemistry in paraffin-embedded tissue section s was positive for the pan T-cell marker MT1, weakly positive for UCHL 1, and negative for CD3, and B-cell markers were negative; these findi ngs were consistent with the diagnosis of T-cell lymphoma. The clinica l, histological and immunological presentation of this lymphoma was si milar to that of hepatosplenic gamma delta T-cell lymphoma. Autoimmune hemolytic anaemia preceded the lymphoma. Despite chemotherapy, the pa tient died 24 months after the initial presentation in the leukemic ph ase. A better understanding of this exceptional but characteristic ent ity is required for an accurate and early diagnosis.