Yonsei Med J.  2008 Aug;49(4):601-609. 10.3349/ymj.2008.49.4.601.

Primary Systemic Anaplastic Large Cell Lymphoma in Korean Adults: 11 Years' Experience at Asan Medical Center

• Park SJ
• Kim S
• Lee DH
• Jeong YP
• Bae Y
• Han EM
• Huh J
• Suh C

• KMID: 1793195
• DOI: http://doi.org/10.3349/ymj.2008.49.4.601

Abstract

PURPOSE
Anaplastic large cell lymphoma (ALCL), a CD30+ T-cell non-Hodgkin's lymphoma, represents only 2-8% of lymphoma overall. Information on the clinical findings of primary systemic ALCL in Korea is limited. Our aims were to report the clinical features and outcomes of primary systemic ALCL. PATIENTS and METHODS: We retrospectively reviewed the medical records of 36 adult patients diagnosed with primary systemic ALCL at Asan Medical Center from February 1995 through June 2006. RESULTS: Of 36 patients, 29 were male. The median age was 39 years (range, 17-67 years), and 26 (72%) presented with Ann Arbor stages III and IV. The most commonly involved extranodal sites were bone (n = 7) and soft tissue (n = 6). Thirty-two of all patients (89%) were treated with an anthracycline-based regimen including cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) as induction chemotherapy; 16 (50%) achieved complete remission (CR), and 13 (41%) achieved partial remission (PR). Median overall survival (OS) and event-free survival (EFS) were 49 and 17 months, respectively. Univariate analysis showed that performance status (p = 0.035), international prognostic index (IPI) (p = 0.025), and age-adjusted IPI (p = 0.034) were significant prognostic factors for OS, whereas anaplastic lymphoma kinase (ALK) expression did not affect OS (p = 0.483). CONCLUSION: Our retrospective analysis of Korean primary systemic ALCL patients showed that median OS was 49 months and overall response to CHOP was 91%. Performance, IPI, and age-adjusted IPI were predictors of OS, whereas ALK expression did not have prognostic significance.

Keyword

Anaplastic large cell lymphoma; anaplastic lymphoma kinase; CD 30; prognosis; International Prognostic Index

MeSH Terms

Adolescent
Adult
Aged
Disease-Free Survival
Female
*Hospitals
Humans
Korea/epidemiology
Lymphoma, Large-Cell, Anaplastic/enzymology/epidemiology/*pathology/therapy
Male
Middle Aged
Neoplasm Staging
Protein-Tyrosine Kinases/metabolism
Survival Rate
Time Factors

Figure

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  Fig. 1 OS and EFS of 36 patients with primary systemic ALCL (median 49.0 and 17.0 months; 5-years survival rate 45 and 15%, respectively). OS, overall survival; EFS, event-free survival.

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  Fig. 2 OS according to performance and International Prognostic Index in 36 patients. OS, overall survival; IPI, International Prognostic Index.

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  Fig. 3 OS and EFS of 30 patients with ALK+ and ALK- primary systemic ALCL (median 17.0 months; 2-year EFS rate 45%; 5-year EFS rate 15%). OS, overall survival; EFS, event-free survival; ALK, anaplastic lymphoma kinase; ALCL, anaplastic large cell lymphoma.

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