Primary Systemic Anaplastic Large Cell Lymphoma in Korean Adults; Retrospective Analysis of 36 Patients.

Anaplastic large cell lymphoma (ALCL) is a CD30+ T-cell non-Hodgikin’s lymphoma and represents only 2–8% of lymphoma overall. Information on clinical finding and response to treatment of primary systemic ALCL is still limited in Korea. The aim of this study was to report our experience thirty-six cases with focusing on the clinical feature, treatment response, survival, and prognosis. We retrospectively reviewed the medical records of the patients who were diagnosed as primary systemic ALCL at the Asan Medical Center from February 1995 through December 2005. The median follow up period was 47 months. The median age was 39 years (range, 17–67). The ratio of male to female was 4.14. Patients with primary systemic ALCL had high frequency of advanced-stage disease and extranodal involvement. Seventy-two percent of patients showed Ann Arbor stage III and IV. The most common involved extranodal sites were bone (19%) and soft tissue (17%). Thirty-two (89%) patients were treated by anthracycline-based regimen (CHOP) as induction chemotherapy. Sixteen patients (44%) obtained CR and 14 patients (39%) attained PR, with overall response rate of 83%. The overall survival and disease-free survival was 49 and 17 months. In ALK-positive patients, median overall survival was not reached in follow up period and 3-year overall survival was 61%. In ALK-negative patients, median overall survival was 53 months and 1-year and 3-year overall survival was 75% and 40% respectively. On univariate analysis, international prognostic index (IPI) was significant prognostic factor for overall survival (p = .03). But ALK expression did not affect overall survival in Korea (p = .78). There was no independent prognostic factor for overall survival in multivariate analysis. This retrospective analysis contributes to our understanding of clinical aspects for patients with primary systemic ALCL in Korea. Overall survival is 49 months and overall response to CHOP chemotherapy is 83%. IPI is important predictor of overall survival, but ALK expression may not provide prognostic information for primary systemic ALCL in Korea.