Krishnan Biju, Matutes Estella, Dearden Claire
Department of Haemato-Oncology, The Royal Marsden Hospital and Institute of Cancer Research, London, UK.
Semin Oncol. 2006 Apr;33(2):257-63. doi: 10.1053/j.seminoncol.2006.01.015.
T and B subtypes of prolymphocytic leukemias (PLLs) are rare, highly aggressive lymphoid malignancies with characteristic morphologic, immunophenotypical, cytogenetic, and molecular features. Recent studies have highlighted the role of specific oncogenes such as TCL1, MTCP-1, and ATM in the case of T-cell and p53 mutations in the case of B-cell PLLs. Despite the advances in the understanding of the biology of these conditions, prognosis for these patients remains poor with short survival and no curative treatment. The advent of monoclonal antibody therapy has improved treatment options for this group. In particular, the use of Campath-1H, in T-PLL has more than doubled median survival. The role of allogeneic transplant with nonmyeloablative conditioning needs to be explored further.
原淋巴细胞白血病(PLL)的T和B亚型是罕见的、侵袭性很强的淋巴恶性肿瘤,具有特征性的形态学、免疫表型、细胞遗传学和分子特征。最近的研究突出了特定癌基因的作用,如T细胞PLL中的TCL1、MTCP-1和ATM,以及B细胞PLL中的p53突变。尽管在对这些疾病生物学的理解方面取得了进展,但这些患者预后仍然很差,生存期短且无治愈性治疗方法。单克隆抗体疗法的出现改善了该群体的治疗选择。特别是,在T-PLL中使用Campath-1H使中位生存期增加了一倍多。非清髓性预处理的异基因移植的作用需要进一步探索。