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加拿大一家三级癌症中心成年T细胞白血病/淋巴瘤患者的独特特征和社会决定因素。

Distinct characteristics and social determinants in adult T-cell leukaemia/lymphoma patients at a tertiary cancer centre in Canada.

作者信息

Aoki Tomohiro, Salib Mary, Suleman Adam, Ghanem Noorhan, Hong Michael, Boriano Agustina, Vijenthira Abi, Prica Anca, Kuruvilla John, Kukreti Vishal, Crump Michael, Kridel Robert

机构信息

Princess Margaret Cancer Centre-University Health Network, Toronto, Ontario, Canada.

出版信息

Br J Haematol. 2025 Jul;207(1):132-140. doi: 10.1111/bjh.20132. Epub 2025 May 6.

DOI:10.1111/bjh.20132
PMID:40328512
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12234277/
Abstract

Adult T-cell leukaemia/lymphoma (ATLL) is a rare, aggressive haematological malignancy linked to human T-cell leukaemia virus type I (HTLV-1) and associated with poor outcomes. Despite its higher prevalence in HTLV-1-endemic regions, the relationship between clinical characteristics and patients' sociocultural background remains underexplored. We retrospectively analysed 79 ATLL patients treated at our institution (1993-2023). The median age at diagnosis was 47 years, and 72% of patients were of Caribbean origin. Median progression-free and overall survival were 10.2 and 16.2 months, respectively, with only five patients receiving allogeneic stem cell transplantation. Central nervous system (CNS) involvement at diagnosis occurred in 22% of patients and was associated with worse outcomes, while 14% experienced CNS relapse within a median of 4.9 months. Using the Ontario Marginalization Index, we found higher levels of material, household/dwelling and racialized/newcomer-related marginalization compared to the general Greater Toronto Area population, though these factors were not linked to poorer outcomes. Our findings reveal that ATLL patients in this cohort were predominantly of Caribbean descent, presented at a young age and faced significant CNS involvement and poor survival outcomes, underscoring ATLL as an unmet clinical need.

摘要

成人T细胞白血病/淋巴瘤(ATLL)是一种罕见的侵袭性血液系统恶性肿瘤,与I型人类T细胞白血病病毒(HTLV-1)相关,预后较差。尽管在HTLV-1流行地区其患病率较高,但临床特征与患者社会文化背景之间的关系仍未得到充分探索。我们回顾性分析了在我们机构接受治疗的79例ATLL患者(1993 - 2023年)。诊断时的中位年龄为47岁,72%的患者来自加勒比地区。无进展生存期和总生存期的中位数分别为10.2个月和16.2个月,只有5例患者接受了异基因干细胞移植。22%的患者在诊断时出现中枢神经系统(CNS)受累,且与较差的预后相关,而14%的患者在中位4.9个月内出现CNS复发。使用安大略边缘化指数,我们发现与大多伦多地区普通人群相比,这些患者在物质、家庭/居住以及与种族化/新移民相关的边缘化方面程度更高,尽管这些因素与较差的预后无关。我们的研究结果表明,该队列中的ATLL患者主要为加勒比裔,发病年龄较轻,面临显著的CNS受累和较差的生存结果,突出了ATLL作为一种未满足的临床需求。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af1f/12234277/900129591908/BJH-207-132-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af1f/12234277/6f6672171f4e/BJH-207-132-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af1f/12234277/e907ac9ff597/BJH-207-132-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af1f/12234277/900129591908/BJH-207-132-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af1f/12234277/6f6672171f4e/BJH-207-132-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af1f/12234277/e907ac9ff597/BJH-207-132-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af1f/12234277/900129591908/BJH-207-132-g003.jpg

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本文引用的文献

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