Savage Kerry J
British Columbia Cancer Agency, Vancouver, BC, Canada.
Hematology Am Soc Hematol Educ Program. 2008:280-8. doi: 10.1182/asheducation-2008.1.280.
Peripheral NK/T-cell neoplasms are an uncommon group of diseases that show distinct racial and geographic variation. The prognostic significance of the T-cell phenotype has been clearly defined in recent studies by using modern lymphoma classification systems. However, within this heterogenous group of neoplasms, some have a more favorable prognosis, such as ALK-positive anaplastic large-cell leukemia (ALCL) and primary cutaneous ALCL, and some have ultimately fatal courses with standard chemotherapy programs (e.g., hepatosplenic gammadelta T-cell lymphomas). Further, unlike the benefits observed with CHOP chemotherapy in the treatment of diffuse large B-cell lymphoma (DLBCL), peripheral T-cell lymphomas (PTCL), other than ALK-positive ALCL, are relatively chemoresistant to this regimen. Given disease rarity and biological heterogeneity, advances in diagnosis, prognosis and treatment have lagged behind DLBCL. Recently, however, studies are emerging that focus specifically on PTCLs with the ultimate goal of better understanding disease biology and developing more effective therapies.
外周NK/T细胞肿瘤是一组罕见的疾病，呈现出明显的种族和地理差异。利用现代淋巴瘤分类系统，T细胞表型的预后意义在最近的研究中已得到明确界定。然而，在这一异质性肿瘤群体中，一些预后较好，如ALK阳性间变性大细胞白血病（ALCL）和原发性皮肤ALCL，而一些采用标准化疗方案最终会导致致命病程（如肝脾γδT细胞淋巴瘤）。此外，与CHOP化疗在弥漫性大B细胞淋巴瘤（DLBCL）治疗中观察到的益处不同，除ALK阳性ALCL外，外周T细胞淋巴瘤（PTCL）对该方案相对化疗耐药。鉴于疾病罕见性和生物学异质性，诊断、预后和治疗方面的进展落后于DLBCL。然而，最近出现了一些专门针对PTCL的研究，其最终目标是更好地理解疾病生物学并开发更有效的治疗方法。
