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T细胞幼淋巴细胞白血病

T-cell prolymphocytic leukemia.

作者信息

Khot Amit, Dearden Claire

机构信息

Department of Hemato-Oncology, Royal Marsden Hospital, London, UK.

出版信息

Expert Rev Anticancer Ther. 2009 Mar;9(3):365-71. doi: 10.1586/14737140.9.3.365.

Abstract

T-cell prolymphocytic leukemia is a rare post-thymic lymphoid disorder, which has distinctive clinical, morphologic, immunophenotypic and cytogenetic features. It has previously been associated with an aggressive course, poor response to conventional chemotherapy and a short median survival. Treatment with purine analogs and the monoclonal antibody alemtuzumab has resulted in significantly higher response rates and increased survival. However, responses are transient and allogeneic hematopoietic progenitor-cell transplantation remains the only potential curative option. The proportion of patients eligible for transplant is low, owing to the older age group of patients, and nonmyeloablative transplantation is a promising alternative that needs to be explored.

摘要

T细胞幼淋巴细胞白血病是一种罕见的胸腺后淋巴样疾病,具有独特的临床、形态学、免疫表型和细胞遗传学特征。它既往与侵袭性病程、对传统化疗反应不佳及中位生存期短相关。嘌呤类似物和单克隆抗体阿仑单抗治疗已使缓解率显著提高且生存期延长。然而,缓解是短暂的,异基因造血祖细胞移植仍是唯一潜在的治愈选择。由于患者年龄较大,适合移植的患者比例较低,非清髓性移植是一种有前景的替代方法,有待探索。

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