[Primary nasopharyngeal lymphoma with CD3- and CD56+ phenotype]

A 73-year-old man was admitted to our hospital with nasal hemorrhage and high grade fever on Aug, 1992. Physical examination revealed a tumor in the nasopharyngeal cavity, generalized skin eruptions and three tumors on different subcutaneous lesions, splenomegaly 2 cm below the costal margin, and the enlargement of the right cervical and axillary lymph nodes. Biopsy of the nasopharyngeal and cutaneous tumor disclosed non-Hodgkin's lymphoma (WF: Diffuse small cleaved). Peripheral blood examination showed a WBC of 4,800/microliters with 10% blastoid cells. Bone marrow examination showed 60% blastoid cells which frequently appeared a hand mirror configuration had no azurophilic granules in the cytoplasm. Flow cytometic analysis of these cells in the bone marrow showed that they expressed CD56 (NKH-1) and Ia but not expressed T-cell antigens as well as B-cell antigens and myeloid cell antigens. Phenotype of subcutaneous tumor biopsy cells was similar to that of blastoid cells in the bone marrow. T-cell receptor gene (TCR beta and gamma) rearrangements in blastoid cells were not found. The patient was treated with local radiotherapy to nasopharyngeal and skin tumors, followed by chemotherapy. The patient died of complication with pulmonary bleeding due to DIC. These results suggested that this nasopharyngeal lymphoma derived from NK cell.