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 primary sinusoidal lymphoma of the liver, in a 36 year-old male patient, revealed by homogeneous hepatosplenomegaly and infiltration of liver sinusoids by morphologically normal lymphocytes, without destruction of the parenchyma. Immunohistochemistry in paraffin-embedded tissue sections was positive for the pan T-cell marker MTI, weakly positive for UCHLI, and negative for CD3, and B-cell markers were negative; these findings were consistent with the diagnosis of T-cell lymphoma. The clinical, histological and immunological presentation of this lymphoma was similar to that of hepatosplenic gamma delta T-cell lymphoma. Autoimmune hemolytic anaemia preceded the lymphoma. Despite chemotherapy, the patient died 24 months after the initial presentation in the leukemic phase. A better understanding of this exceptional but characteristic entity is required for an accurate and early diagnosis.
Pouderoux
P
P
Service d'Hépato-Gastroentérologie et Alcoologie, Centre Hospitalier Universitaire, Nimes.
Gris
J C
JC
Pignodel
C
C
Joujoux
J M
JM
Schved
J F
JF
Balmes
J L
JL
D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008113:Liver Neoplasms / Q000150:complications* / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000473:pathology; D008297:Male