Gamma/delta T cell lymphoma presenting in the subcutaneous tissue and small intestine in a patient with capillary leak syndrome.

Peripheral T cell neoplasms originating from gamma/delta T cells are rarely reported, despite the development of hepatosplenic lymphoma from gamma/delta T cells. We report a case of multiple generated gamma/delta T cell lymphomas presenting in the subcutaneous tissue and small intestine in a patient with accompanying capillary leak syndrome. The patient, a 43-year-old male presented with remarkable systemic edema, pleural effusion, and ascites. Widespread tumors were evident in the subcutaneous tissue and in the small intestine. A biopsy revealed anaplastic large cell lymphoma in these lesions. Phenotypic examination showed that the neoplastic cells were positive for the surface markers CD3 and TCR delta, but negative for CD4, CD8 and TCR beta. Genomic analysis revealed a clonal rearrangement of the TCR genes encoding the gamma, delta, and beta chains. A polymerase chain reaction analysis of the gene encoding the TCR delta chain showed that the rearrangement occurred between V delta 3 and J delta 1, suggesting that neoplastic cells were generated in the early stages of T cell differentiation.