A case of blastic natural killer cell lymphoma with rapidly progressive multi-organ involvement

Abstract

The clinical history of a 72-year old male with blastic NK-cell lymphoma is reported. Initial manifestation consisted of skin infiltrations and axillary lymphomas. The patient was treated with cyclophosphamide, doxorubicine, vincrisitine, and prednisone (CHOP), resulting in a partial response lasting for two months. First relapse presented with splenomegaly, leukaemic generalisation, and multifocal cutaneous involvement. Under treatment with dexamethasone and cyclophosphamide, rupture of the enlarged spleen caused haematoperitoneum. After emergency splenectomy, thrombocytopenia refractory to prior cytoreductive therapy resolved spontaneously. Chemotherapy was resumed with drugs commonly used in induction therapy of acute lymphocytic leukaemia (ALL) (standard-dosed cytosinearabinoside, cyclophosphamide, and steroids) but yielded no effect. Response to dexamethasone, carmustine, etoposide, doxorubicine, and melphalan (DexaBEAM) lasted for six weeks. A meningeal relapse was treated with high-dose methotrexate and blast clearance from the cerebrospinal fluid and a temporary partial remission of peripheral manifestations could be achieved. The patient finally succumbed to bleeding from progressive lymphatic infiltration of the upper aerodigestive tract nine months after diagnosis. NK-cell lymphomas are aggressive lymphatic diseases which characteristically show extra-nodal involvement of various sites of the body. No adequate chemotherapy has been defined so far. Thus, experimental therapy should be considered early in the course of disease.