Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Department of Oncology, Jiangsu Province Hospital of Traditional Chinese Medicine, Nanjing, Jiangsu, China.
Am J Hematol. 2017 May;92(5):441-447. doi: 10.1002/ajh.24679. Epub 2017 Feb 27.
T-cell prolymphocytic leukemia (T-PLL) is an aggressive mature T-cell neoplasm. The most common cytogenetic abnormality associated with T-PLL is inv(14)(q11.2q32) involving TCL1, but other abnormalities also have been reported. In this study, we correlated cytogenetic abnormalities with clinical outcome in 97 T-PLL patients, including 66 men and 31 women with a median age of 63 years (range, 34-81). Twenty-seven patients had a normal karyotype (NK), one had two chromosomal aberrations, and 69 had a complex karyotype (CK). Patients with a CK had poorer overall survival (OS) than patients with a NK (P = .0016). In the CK group, the most common aberrations involved 14q (n = 45) and 8q (n = 38). Additional deletions of chromosomes 17p, 11q, 6q, 12p, 13q were observed frequently. No individual cytogenetic abnormality impacted OS. Patients with ≥5 aberrations had an OS of 11 months versus 22 months in patients with <5 aberrations (P = 0.0132). Fluorescence in situ hybridization for TCL1 successfully performed in 27 cases showed rearrangement in 8/10 (80%) NK versus 16/17 (94%) CK cases. OS of patients with TCL1 rearrangement and/or 14q aberrations was not significantly different from patients without TCL1 rearrangement and 14q aberrations (P = .3467). Patients with refractory disease showed worse OS in both the NK and CK groups (P = .0014 and P < .0001, respectively), compared with patients who achieved remission but then relapsed. Stem cell transplantation did not appear to improve OS regardless of karyotype complexity. In conclusion, patients with T-PLL often have a CK which is a poor prognostic factor, particularly in patients with ≥5 cytogenetic aberrations.
T 细胞前淋巴细胞白血病（T-PLL）是一种侵袭性成熟 T 细胞肿瘤。与 T-PLL 相关的最常见细胞遗传学异常是 inv(14)(q11.2q32)，涉及 TCL1，但也有其他异常报道。在这项研究中，我们将 97 例 T-PLL 患者的细胞遗传学异常与临床结果相关联，包括 66 名男性和 31 名女性，中位年龄为 63 岁（范围为 34-81 岁）。27 例患者核型正常（NK），1 例患者有两种染色体异常，69 例患者核型复杂（CK）。CK 组患者的总生存（OS）明显差于 NK 组患者（P=.0016）。在 CK 组中，最常见的异常涉及 14q（n=45）和 8q（n=38）。还经常观察到 17p、11q、6q、12p、13q 的额外缺失。没有单个细胞遗传学异常影响 OS。有≥5 个异常的患者 OS 为 11 个月，而有<5 个异常的患者 OS 为 22 个月（P=.0132）。成功进行了 27 例 TCL1 荧光原位杂交，显示 10 例中的 8 例（80%）NK 患者和 17 例中的 16 例（94%）CK 患者存在重排。TCL1 重排和/或 14q 异常患者的 OS 与无 TCL1 重排和 14q 异常患者的 OS 无显著差异（P=.3467）。在 NK 和 CK 组中，难治性疾病患者的 OS 均较差（P=.0014 和 P<.0001），与获得缓解但随后复发的患者相比。无论核型复杂性如何，干细胞移植似乎都不能改善 OS。总之，T-PLL 患者常有 CK，这是一个不良预后因素，特别是在有≥5 个细胞遗传学异常的患者中。
