Definition, Prognostic Factors, Treatment, and Response Criteria of Adult T-Cell Leukemia-Lymphoma: A Proposal From an International Consensus Meeting
Definition, Prognostic Factors, Treatment, and Response Criteria of Adult T-Cell Leukemia-Lymphoma: A Proposal From an International Consensus Meeting
复制标题
DOI：
10.1200/jco.2008.18.2428
复制
发表时间：
2009-01-20
影响因子：
45.3
通讯作者：
Watanabe, Toshiki
中科院分区：
文献类型：
--
作者：
Tsukasaki, Kunihiro;Hermine, Olivier;Watanabe, Toshiki
成人 T 细胞白血病淋巴瘤 (ATL) 是一种独特的外周 T 淋巴细胞恶性肿瘤，与 I 型人类 T 细胞嗜淋巴细胞病毒 (HTLV-1) 逆转录病毒相关。该疾病患者临床特征和预后的多样性导致其细分为以下四类：急性型、淋巴瘤型、慢性型和阴燃型。慢性和冒烟亚型被认为是惰性的，通常通过观察等待直至疾病进展进行治疗，类似于一些慢性淋巴细胞白血病（CLL）或其他惰性组织学淋巴瘤患者的治疗。侵袭性 ATL 患者通常预后较差，因为恶性细胞具有多药耐药性、肿瘤负荷大且多器官衰竭、高钙血症和/或严重 T 细胞免疫缺陷导致的频繁感染并发症。在第 13 届人类逆转录病毒学国际会议：HTLV 的赞助下，一组 ATL 研究人员根据既定数据形成了共识声明，以定义预后因素、临床亚分类和治疗策略。提出了一套针对 ATL 的反应标准，反映了淋巴瘤和 CLL 的组合。临床亚分类是有用的，但由于每种亚型的预后不同而受到限制。宿主基因组内的分子异常，例如肿瘤抑制基因，可能是这些多样性的原因。建议基于临床亚分类和预后因素的治疗策略，包括观察等待方法、化疗、抗病毒治疗、同种异体造血干细胞移植（alloHSCT）和靶向治疗。
Adult T-cell leukemia-lymphoma ( ATL) is a distinct peripheral T-lymphocytic malignancy associated with a retrovirus designated human T-cell lymphotropic virus type I (HTLV-1). The diversity in clinical features and prognosis of patients with this disease has led to its subclassification into the following four categories: acute, lymphoma, chronic, and smoldering types. The chronic and smoldering subtypes are considered indolent and are usually managed with watchful waiting until disease progression, analogous to the management of some patients with chronic lymphoid leukemia (CLL) or other indolent histology lymphomas. Patients with aggressive ATL generally have a poor prognosis because of multidrug resistance of malignant cells, a large tumor burden with multiorgan failure, hypercalcemia, and/or frequent infectious complications as a result of a profound T-cell immunodeficiency. Under the sponsorship of the 13th International Conference on Human Retrovirology: HTLV, a group of ATL researchers joined to form a consensus statement based on established data to define prognostic factors, clinical subclassifications, and treatment strategies. A set of response criteria specific for ATL reflecting a combination of those for lymphoma and CLL was proposed. Clinical subclassification is useful but is limited because of the diverse prognosis among each subtype. Molecular abnormalities within the host genome, such as tumor suppressor genes, may account for these diversities. A treatment strategy based on the clinical subclassification and prognostic factors is suggested, including watchful waiting approach, chemotherapy, antiviral therapy, allogeneic hematopoietic stem-cell transplantation (alloHSCT), and targeted therapies.