Hepatobiliary and Pancreatic: Hepatosplenic gamma‐delta T‐cell lymphoma

Abstract

Hepatosplenic gamma-delta (gd) T-cell lymphoma is a rare form of peripheral T-cell lymphoma that was first proposed as a distinct clinicopathologic entity in 1990. Common presenting symptoms include fever, weakness and abdominal pain. On examination, the liver and spleen are enlarged but there are no enlarged peripheral lymph nodes. Typical blood tests reveal anemia, leukopenia and thrombocytopenia but liver function tests are either normal or near-normal. Histological features are characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen and sinuses of the bone marrow. Immunophenotypic expressions include CD2 + , CD3 + , CD4 , CD5, CD7, CD16, CD56, TIA-1 + , TdT and granzyme B. The T-cell receptor—gd phenotype (TCRd1 + ) is positive while the T-cell receptor—ab phenotype (bF1) is negative. Thus far, responses to chemotherapeutic regimens have been poor and patients have a median survival of only 11‐16 months. Of interest to gastroenterologists are case reports of hepatosplenic gd T-cell lymphomas in patients with inflammatory bowel disease after treatment with monoclonal antibodies against tumor necrosis factor. Most of these patients have been young men receiving concomitant medication with azathioprine, 6-mercaptopurine or methotrexate. The patient illustrated below was a 46-year-old man who was admitted to hospital with upper abdominal distension and peripheral edema. Twelve months previously, he had been treated by splenectomy for splenomegaly and features of hypersplenism. An abdominal computed tomography scan showed a huge liver that extended into the pelvis. The liver had a heterogeneous appearance but no discrete nodules (Figure 1). Blood tests revealed mild anemia (hemoglobin 108 g/l), a mild elevation of the white cell count (13.4x10 9