Natural killer cell lymphoma - Report of two pediatric cases, therapeutic options, and review of the literature

BACKGROUND. Natural killer (NK) cell lymphomas are rapidly fatal malignanci es that to the authors' knowledge are rare in children In the current study , the authors report me cases or two boys with NK cell lymphomas with refra ctory disease who both were salvaged with high dose chemotherapy and stem c ell transplantation and compare these patients with those in the published experience. authors report the cases of two boys with NK cell lymphomas wit h refractory METHODS. A comprehensive literature review was performed to identify other cases of pediatric patients with NX cell lymphomas, their treatment, and ou tcome. RESULTS. One of the patients in the current study developed two recurrences and the other patient experienced early disease progression during front-l ine treatment. Both then were treated with high dose chemotherapy followed by stem cell rescue. At last follow-up, the patients remained free of disea se at 15 months and 16 months, respectively, after transplantation (48 mont hs and 22 months, respectively, from the time of diagnosis). In addition to the 2 patients in the current study, the authors found 13 pediatric patien ts reported in the literature to date. Of the 7 patients with localized (St age I-II) disease, 5 patients (71%) were reported to be alive 1-107 months after diagnosis. Of the 6 patients with Stage IV disease, only the 2 patien ts who received high dose chemotherapy and stem cell rescue (33%) were aliv e at the time of last follow-up (at 30 months and 12 months, respectively). Including the patients reported in the current study, 9 of 15 children wit h NK cell lymphoma (all stages) (60%) were reported to be alive at the time of last follow-up. CONCLUSIONS, Although pediatric NK cell lymphomas rapidly can become fatal, it appears that high dose chemotherapy followed by stem cell transplantati on is effective therapy, especially in patients with advanced or resistant disease. Further follow-up is needed to determine whether this treatment ap proach will be curative. (C) 2001 American Cancer Society.