Department of Virology, Faculty of Public Health, Tehran University of Medical Sciences, Tehran, Iran.
Research Center for Clinical Virology, Tehran University of Medical Science, Tehran, Iran.
Med Oncol. 2023 Sep 9;40(10):295. doi: 10.1007/s12032-023-02166-8.
Adult T-cell leukemia/lymphoma (ATLL), an infrequent malignancy resultant from human T-cell lymphotropic virus type I (HTLV-1), exhibits a spectrum of phenotypes, encompassing acute, smoldering, lymphomatous, and chronic variants, each bearing distinct clinical presentations. The preponderant acute manifestation is characterized by hypercalcemia, systemic manifestations, organomegaly, and dermatological eruptions. Conversely, the chronic phenotype is typified by lymphocytosis and/or cutaneous eruptions, while smoldering ATLL assumes an asymptomatic course. Immunocompromise afflicts ATLL patients, heightening their vulnerability to opportunistic infections that frequently intricately intertwine with disease progression. Therefore, an early diagnosis is crucial to manage the disease appropriately. While conventional chemotherapeutic regimens have shown limited success, especially in acute and lymphoma types, recent studies suggest that allogeneic stem cell transplantation might enhance treatment results because it has shown promising outcomes in some patients. Novel therapeutics, such as interferon and monoclonal antibodies, have also shown promise, but more research is needed to confirm their efficacy. Moreover, the identification of biomarkers for ATLL and genetic changes in HTLV-1 infected cells has led to the development of targeted therapies that have shown remarkable success in clinical trials. These targeted therapies have the potential to offer a more personalized approach to the treatment of ATLL. The aim of our review is to elaborate on conventional and novel therapies and the efficiency of mentioned treatments.
成人 T 细胞白血病/淋巴瘤(ATLL)是一种罕见的恶性肿瘤,由人类 T 细胞嗜淋巴细胞病毒 I 型(HTLV-1)引起,表现出多种表型,包括急性、亚急性、淋巴瘤和慢性变异型,每种变异型都具有不同的临床表现。主要的急性表现为高钙血症、全身表现、器官肿大和皮肤疹。相反,慢性表型的特点是淋巴细胞增多和/或皮肤疹,而亚急性 ATLL 呈无症状过程。ATLL 患者免疫功能受损,易发生机会性感染,这些感染常与疾病进展交织在一起。因此,早期诊断对于适当治疗疾病至关重要。虽然传统的化疗方案已经显示出有限的成功,特别是在急性和淋巴瘤类型中,但最近的研究表明,同种异体干细胞移植可能会增强治疗效果,因为它在一些患者中显示出了有希望的结果。新型治疗药物,如干扰素和单克隆抗体,也显示出了希望,但需要更多的研究来确认它们的疗效。此外,ATLL 的生物标志物和 HTLV-1 感染细胞的遗传变化的鉴定导致了靶向治疗的发展,这些治疗在临床试验中取得了显著的成功。这些靶向治疗有可能为 ATLL 的治疗提供更个性化的方法。我们的综述旨在详细阐述传统和新型治疗方法以及上述治疗方法的效果。