Risk stratification in extranodal natural killer/T-cell lymphoma.
ABSTRACT Extranodal natural killer/T-cell lymphoma (ENKL), a subtype of natural killer/T-cell malignancies, is a rare subset of lymphomas with significant biological and clinical heterogeneity. The prognosis of ENKL is variable and therapeutic approaches are not well established. The optimal dose, combination, and sequence of radiotherapy and chemotherapy are evolving, as is the role of stem cell transplantation. Radiotherapy is an essential component of therapy for early-stage disease. The clinical course of advanced disease is highly aggressive, with frequent chemotherapy resistance and a poor prognosis. For relapsed disease, asparaginase-based regimens have provided encouraging results and are currently under investigation in the frontline setting. Our article discusses the key aspects of biology, pathogenesis and clinical presentation that contribute to the heterogeneity, and proposes a stratified approach to the treatment of ENKL based on clinical, pathologic and biologic risk factors. Although considerable advances have been made in our understanding of the biology and prognosis of this lymphoma, it remains critical that all patients with a diagnosis of ENKL are enrolled and treated in clinical trials so that optimal therapies can be identified.
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ABSTRACT: Extranodal lymphomas which present in the nasal cavity and/or the paranasal sinuses are rare. Thirty-eight patients with disease that was clinically limited to the head and neck (Ann Arbor Stages IE-IIE) were admitted between 1947 and 1983. Twenty-eight patients were treated with radiotherapy alone and 10 received combination chemotherapy in addition. The overall 5-year survival figure was 56%. The corresponding result for Stage IE was 67%. No patient with Stage IIE disease survived 5 years. Extent of the extranodal disease also influenced results for Stage IE patients who were treated with radiotherapy only. When the extranodal disease was staged using the American Joint Committee TNM system, the 5-year disease-free survival for T1 and T2 patients was 78% as compared with 19% for patients with T3 and T4 disease. The addition of combination chemotherapy improved results for patients with T3 and T4 lesions.Cancer 09/1985; 56(4):814-9. · 5.20 Impact Factor
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ABSTRACT: To report our experience managing a large series of Chinese patients with primary nasal lymphoma. From January 1975 to December 1993, 100 patients (median age, 50 years) with newly diagnosed primary nasal lymphoma were studied. There were four low-grade, 62 intermediate-grade, nine high-grade, and 25 unclassifiable lymphomas. Immunophenotyping was performed in 45 patients: eight B cell, 35 T cell, and two uncertain. All cases of angiocentric lymphoma that were typed were T cell. Fifty-two patients had stage I disease, 15 had stage II, four had stage III, and 29 had stage IV. Only 15 patients had B symptoms (weight loss, night sweats, and/or fever), and 11 had bulky disease. Thirty-nine patients with clinically localized stage I and II disease received local radiotherapy alone (before 1980), and the remaining 28 stage I and II patients received combination chemotherapy followed by local radiotherapy. The 33 patients with advanced stage III and IV disease were given combination chemotherapy, and additional radiotherapy was given to five of them who had bulky local disease. Significantly higher complete remission rates were observed in patients with early stages of disease and those without B symptoms. Superior disease-free survival after complete remission was observed in patients with stage I/II disease. Univariate factors associated with a better overall survival included age less than 60 years, stage I disease, and absence of B symptoms. Survival was significantly better in the subgroup of patients with stage I disease. Patients with nasal lymphoma, especially those with advanced disease, seemed to have a poor prognosis, and their clinical outcome was not improved significantly by the use of chemotherapy instead of radiotherapy or the use of doxorubicin-containing chemotherapeutic regimens.Journal of Clinical Oncology 04/1995; 13(3):666-70. · 18.04 Impact Factor
- The Lancet 05/1996; 347(9008):1124. · 39.06 Impact Factor