Aggressive NK cell lymphoma preceded by a ten year history of neutropenia associated with large granular lymphocyte lymphocytosis

We report a case of aggressive natural killer (NK) cell lymphoma in an 82 y ear old man who first presented 10 years earlier with neutropenia in associ ation with a large granular lymphocyte (LGL) lymphocytosis. The diagnosis o f NK cell lymphoma was made on the basis of morphological and immunological characteristics (CD3-CD56+) found on skin biopsy of one of multiple skin n odules which subsequently developed in association with splenomegaly, throm bocytopenia and continuing neutropenia. In addition there was BM infiltrati on and a cytogenetic abnormality [add(6)(p25)] was detected. Combination ch emotherapy led to an initial clinical response but a relapse occurred short ly afterwards and the patient died 8 months later from infection whilst neu tropenic following re-introduction of chemotherapy, Previously reported cas es of aggressive NK cell lymphoma have shown a young male predominance with a rapidly progressive clinical course and without evidence of a preceding chronic phase of LGL lymphocytosis and neutropenia.