Diagnosis and Management of Natural Killer-cell Malignancies

Fumihiro Ishida; Yok-Lam Kwong

Disclosures

Expert Rev Hematol. 2010;3(5):593-602. 

In This Article

Treatment of Advanced-stage NK-cell Lymphoma (Nasal & Non-nasal)

Although chemotherapy is the sole approach for the treatment of advanced-stage NK-cell lymphomas, CHOP-based therapy has demonstrated a dismal outcome.[19,27] CR was achieved at a rate of only 20% or less and few patients survived. It was considered doubtful whether cure with chemotherapy was possible.

A new therapeutic regimen consisting of dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide (SMILE regimen; Table 2) has given promising results in Phase I and Phase II studies.[47,48] The concept of SMILE therapy is based on several lines of in vitro and in vivo data against NK-lymphoma cells, in which the order and co-administration of these drugs might exert beneficial effects. Approximately half of the patients treated with this regimen were in stage IV disease. The treatment outcome was remarkable, with an OR rate of 74%, and a CR rate of 35–50%.[47,48] Neutropenia and infections were common adverse effects, necessitating vigorous granulocyte-colony stimulating factor support. The long-term outcome of these patients remains to be defined.

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