State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.
Department of Pathology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.
Front Med. 2015 Dec;9(4):468-77. doi: 10.1007/s11684-015-0419-6. Epub 2015 Nov 11.
Mature T-cell lymphoid malignancies comprise a group of heterogeneous diseases that vary in clinicopathological features, biological behavior, treatment response, and prognosis. Bone marrow (BM) infiltration is more commonly present in mature T-cell lymphoid malignancies compared with their B-cell counterparts and hence important for differential diagnosis. In this study, clinical characteristics and prognostic factors were analyzed in 225 patients with mature T-cell lymphoid malignancies treated in a single institution. These included 29 cases of T-cell lymphoproliferative disorders (T-LPD, all with BM infiltration) and 196 cases of T-/natural-killer-cell lymphoma (T/NKCL, 56 with BM infiltration and 140 without BM infiltration). The estimated 5-year overall survival (OS) rates of T-LPD and T/NKCL were 96.6% and 37.3%, respectively. T-LPD patients were less likely to exhibit poor performance status, advanced disease stage, presence of B symptoms, or abnormal level of serum β-2 microglobulin. With similar pathological characteristics, T/NKCL patients with BM infiltration showed significantly lower response rates and shorter OS than those without BM infiltration (P = 0.0264 and P < 0.0001, respectively). Multivariate analysis indicated that poor performance status, advanced disease stage, elevated serum lactate dehydrogenase level, and BM involvement were independent unfavorable prognostic factors. The Glasgow Prognostic Score may be more efficient than the International Prognostic Index in predicting disease outcome in T/NKCL. In conclusion, clinical characteristics may be useful in more effectively stratifying patients with mature T-cell lymphoid malignancies.
成熟 T 细胞淋巴恶性肿瘤包括一组具有不同临床病理特征、生物学行为、治疗反应和预后的异质性疾病。与 B 细胞恶性肿瘤相比，成熟 T 细胞淋巴恶性肿瘤更常发生骨髓浸润，因此对鉴别诊断很重要。在这项研究中，分析了在一家机构治疗的 225 例成熟 T 细胞淋巴恶性肿瘤患者的临床特征和预后因素。这些患者包括 29 例 T 细胞淋巴增生性疾病（T-LPD，均有骨髓浸润）和 196 例 T/NK 细胞淋巴瘤（T/NKCL，56 例有骨髓浸润，140 例无骨髓浸润）。T-LPD 和 T/NKCL 的 5 年总生存率（OS）估计分别为 96.6%和 37.3%。T-LPD 患者更不可能表现出较差的体能状态、晚期疾病分期、B 症状存在或血清β-2 微球蛋白水平异常。具有相似病理特征的 T/NKCL 患者，骨髓浸润与较低的缓解率和较短的 OS 相关（P=0.0264 和 P<0.0001）。多因素分析表明，较差的体能状态、晚期疾病分期、血清乳酸脱氢酶水平升高和骨髓受累是独立的不良预后因素。格拉斯哥预后评分可能比国际预后指数更能有效地预测 T/NKCL 患者的疾病结局。总之，临床特征可用于更有效地对成熟 T 细胞淋巴恶性肿瘤患者进行分层。
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