Rasul K I, Barwari Z A
Al-Amal Hospital, Hamad Medical Corporation, Doha, Qatar.
Gulf J Oncolog. 2012 Jul(12):27-37.
The adult T-cell leukemia/lymphoma (ATLL) syndromes comprise neoplasms that arise in peripheral lymphoid tissues but a high frequency present with blood involvement mimicking T-cell leukemia. Clinically ATLL is sub-classified into four groups: acute, lymphomatous, chronic and smoldering. ATLL is etiologically linked to the human T-cell lymphotropic virus type I (HTLV-I). The diagnosis of ATLL is based upon a combination of characteristic clinical manifestations, morphological and immunophenotypic changes of the malignant cells, in addition to the confirmation of HTLV-I infection. ATLL is an aggressive malignancy with a median survival of less than 12 months and no successful treatment yet available. Patients are either refractory or only transiently respond to chemotherapy or purine analogues. Smoldering and chronic ATLL pursue an indolent course and survival for years until the disease progresses and becomes refractory to therapy. The major causes of death in ATLL are opportunistic pulmonary infections and progressive disease, often in association with hypercalcemia.
成人T细胞白血病/淋巴瘤（ATLL）综合征包括起源于外周淋巴组织的肿瘤，但有很高比例会出现血液受累，类似T细胞白血病。临床上，ATLL分为四组：急性、淋巴瘤型、慢性和隐匿型。ATLL在病因上与I型人类嗜T细胞病毒（HTLV-I）有关。ATLL的诊断基于特征性临床表现、恶性细胞的形态学和免疫表型变化的综合判断，此外还需确认HTLV-I感染。ATLL是一种侵袭性恶性肿瘤，中位生存期不到12个月，目前尚无成功的治疗方法。患者对化疗或嘌呤类似物要么耐药，要么仅产生短暂反应。隐匿型和慢性ATLL病程进展缓慢，可存活数年，直到病情进展并对治疗产生耐药。ATLL的主要死亡原因是机会性肺部感染和疾病进展，常伴有高钙血症。
