Aggressive pleomorphic CD2+, CD3-, CD56+ lymphoma with t(5;9)(q31;q34) abnormality

CD56-positive lymphoma is a recently described entity which is characterized by predominantly extranodal involvement and an aggressive clinical course. We report one such case with involvement of the bone marrow and spinal cord at presentation, and associated with reactive hemophagocytic syndrome. The lymphoma cells had a highly pleomorphic appearance which is uncommon in CD56-positive lymphoma. Cytogenetic studies revealed a t(5;9)(q31;q34) abnormality. Analysis of more cases is required to determine if this is a recurring chromosomal translocation characteristic of the group of aggressive CD56-positive lymphoma.