T-CELL LYMPHOMA OF CD3+CD4+CD56+ GRANULAR LYMPHOCYTES WITH HEMOPHAGOCYTIC SYNDROME

A case of T-cell lymphoma of granular lymphocyte (GL) morphology and C D3(+)CD4(+)CD56(+) phenotype is reported. The lymphoma originated in t he subcutaneous tisse of the right thigh and subsequently infiltrated into various organs including the lymph nodes, peripheral blood, bone marrow, skin, lungs, liver, spleen, and peritoneal cavity, and the pat ient finally developed a hemophagocytic syndrome. The lymphoma cells w ere positive for CD2, CD3, CD4, and CD56, and negative for CD8, CD16, CD20, and CD57. An identical clonal rearrangement of the T-cell recept or beta chain genes was detected in cells from a lymph node, periphera l blood, and ascites fluid, indicating the monoclonal proliferation of T cells in such organs. This is a T-cell GL lymphoma, expressing the unusual phenotype of CD3(+)CD4(+)CD8(-)CD16(-)CD56(+), which has not b een previously reported. The development of hemophagocytic syndrome ha s also to our knowledge not previously been reported in T-cell GL lymp homa.