Catovsky D
Royal Marsden Hospital, London, UK.
Curr Opin Oncol. 1995 Jan;7(1):3-11.
There have been important advances in the diagnosis and treatment of chronic lymphoproliferative disorders. Three diseases stand out as examples of progress: chronic lymphocytic leukemia (CLL), hairy-cell leukemia (HCL), and adult T-cell leukemia-lymphoma. Improvements in diagnosis are the result of greater attention to cell morphology and the routine use of monoclonal antibodies, which in turn help define composite phenotypes that are specific for CLL and HCL. The combination of morphology and immunophenotype allowed the characterization of a new disorder, splenic lymphoma with villous lymphocytes, which was confused in the past with both CLL and HCL. The most important treatment developments stem from the availability of three nucleoside analogues: fludarabine, deoxycoformycin, and chlorodeoxyadenosine which, when used as single agents, are responsible for major improvements in remission rates in CLL (fludarabine, chlorodeoxyadenosine), HCL (deoxycoformycin, chlorodeoxyadenosine) and prolonged periods of disease-free survival. Adult T-cell leukemia-lymphoma remains a unique leukemia model, and interest in the worldwide distribution of the causative retrovirus, human T-cell lymphotropic virus I, continues with emphasis on epidemiologic and prevention studies.
慢性淋巴细胞增殖性疾病的诊断和治疗取得了重要进展。有三种疾病是进展的典型例子：慢性淋巴细胞白血病（CLL）、毛细胞白血病（HCL）和成人T细胞白血病-淋巴瘤。诊断方面的进步源于对细胞形态学的更多关注以及单克隆抗体的常规使用，这反过来有助于确定CLL和HCL特有的复合表型。形态学和免疫表型的结合使得一种新的疾病——伴有绒毛状淋巴细胞的脾淋巴瘤得以被描述，这种疾病过去曾与CLL和HCL混淆。最重要的治疗进展源于三种核苷类似物的出现：氟达拉滨、脱氧助间型霉素和氯脱氧腺苷，这些药物单独使用时，可使CLL（氟达拉滨、氯脱氧腺苷）、HCL（脱氧助间型霉素、氯脱氧腺苷）的缓解率得到显著提高，并延长无病生存期。成人T细胞白血病-淋巴瘤仍然是一种独特的白血病模型，对致病逆转录病毒——人类T细胞白血病病毒I在全球分布的研究仍在继续，重点是流行病学和预防研究。
