Gamma-delta hepato-splenic T-cell lymphoma: a pathological illustration

Excerpt

A 57-year-old gentleman with Crohn’s disease on Azathioprine presented with jaundice, liver dysfunction, hepatosplenomegaly, constitutional symptoms, pancytopenia and elevated LDH (9903, normal < 425). Blood film showed several circulating primitive vacuolated cells with voluminous cytoplasm, prominent nucleoli, no visible granules or Auer rods. Staging CT reported 21-cm splenomegaly plus porta-hepatic and subcarinal lymph nodes. Virology (including retrovirus status) was negative. Liver biopsy concluded cholestatic hepatitis with an associated T-cell infiltrate [CD2/CD3/CD7+ve, CD4/CD8/CD56−ve, Ki67 100%] (Fig. 1a, arrowed, tumor cell, intra-sinusoidal; Fig. 1b, CD3 immunostaining, including areas of infiltrating linear cells, in keeping with intra-sinusoidal disease). Bone marrow aspirate (Fig. 1c) and trephine showed a dense infiltration of primitive cells (Fig. 1d; CD3 immunostaining) with a “blastic” appearance, majority vacuolated, immature chromatin with reduced normal tri-lineage hematopoiesis. Bone marrow flow cytometry concluded 60% lymphoid cells, an identical T-cell immunophenotype to liver biopsy, TCR γδ+ve with variable CD7 expression (Fig. 1e). Molecular diagnostics confirmed clonality (TCR γδ rearrangements; bone marrow aspirate; Fig. 1f). Gamma-delta hepato-splenic T-cell lymphoma is a rare disorder, said to be more common in those on immunosuppression, like our patient. This disease is characterized by poor response rates, low treatment tolerability and short survival measurable in months. The few responders are considered for allogeneic transplant. This case demonstrates the benefit of expert integrated diagnostics in an infrequently encountered pathological disorder. The patient was primary refractory to CHOP and ESHAP, succumbing to his disease with progressive liver failure including encephalopathy within 6 months. …