人体T淋巴细胞病毒I型(HTLV-1)引发的成人T细胞白血病/淋巴瘤(ATLL) 是一种罕见的恶性肿瘤，表现为多种表型，包括急性、潜行、淋巴肉瘤和慢性变异，每种变异都具有不同的临床表现。优势的急性表型特征是高钙血症，系统性症状，器官肿大和皮肤疹。相反，慢性表型以淋巴细胞增多和/或皮肤疹为特征，而潜行性ATLL则呈现无症状的过程。ATLL患者免疫功能受损，易于患上机会性感染，并且这些感染往往与疾病进展错综复杂地交织在一起。因此，早期诊断对于适当管理疾病至关重要。尽管常规化疗方案在急性和淋巴瘤类型上显示出有限的成功，但最新研究表明，异基因干细胞移植可能会提高治疗效果，因为在某些患者中已经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，也显示出前景，但还需要进一步研究以确认其有效性。此外，ATLL病人的生物标志物的识别和HTLV-1感染细胞中的遗传变化的发现，已经导致了针对性治疗的开发，这些治疗在临床试验中显示出了显著的成功。这些针对性治疗具有潜力为ATLL的治疗提供更个性化的方法。我们的综述的目的是阐述常规和新型治疗以及提及治疗的效果。© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

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.© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.