Service de Maladies Infectieuses et Tropicales, Centre d'Infectiologie Necker-Pasteur, Hôpital Necker Enfants Malades, Assistance Publique-Hôpitaux de Paris, Université de Paris, Paris, France.
Service d'Hématologie Adultes, Hôpital Necker Enfants Malades, Assistance Publique-Hôpitaux de Paris, Université de Paris, Paris, France.
Ann Hematol. 2021 Nov;100(11):2813-2824. doi: 10.1007/s00277-021-04622-9. Epub 2021 Aug 13.
Patients treated for adult T-Cell leukemia/lymphoma (ATL) have a poor prognosis and are prone to infectious complications which are poorly described. As the French reference center for ATL, we retrospectively analyzed 47 consecutive ATL (acute, n = 23; lymphoma, n = 14; chronic, n = 8; smoldering, n = 2) patients between 2006 and 2016 (median age 51 years, 96% Afro-Caribbean origin). The 3-year overall survival (OS) was 15.8%, 11.3%, and 85.7% for acute, lymphoma, and indolent (chronic and smoldering) forms respectively. Among aggressive subtypes, 20 patients received, as frontline therapy, high dose of zidovudine and interferon alfa (AZT-IFN⍺) resulting in an overall response rate (ORR) of 39% (complete response [CR] 33%) and 17 chemotherapy resulting of an ORR of 59% (CR 50%). Ninety-five infections occurred in 38 patients, most of whom had an acute subtype (n = 73/95; 77%). During their follow-up, patients receiving frontline chemotherapy or frontline AZT-IFNα developed infections in 74% (n = 14/19) and 89% (n = 24/27) of the cases respectively. Sixty-four (67%) of infections were microbiologically documented. Among them, invasive fungal infections (IFI, n = 11) included 2 Pneumocystis jirovecii pneumonia, 5 invasive aspergillosis, and 4 yeast fungemia. IFI exclusively occurred in patients with acute subtype mostly exposed to AZT-IFNα (n = 10/11) and experiencing prolonged (> 10 days) grade 4 neutropenia. Patients with aggressive subtype experiencing IFI had a lower OS than those who did not (median OS 5.4 months versus 18.4 months, p = 0.0048). ATL patients have a poor prognosis even in the modern era. Moreover, the high rate of infections impacts their management especially those exposed to AZT-IFNα.
接受成人 T 细胞白血病/淋巴瘤 (ATL) 治疗的患者预后较差，容易发生感染并发症，但这些并发症描述得并不充分。作为 ATL 的法国参考中心，我们回顾性分析了 2006 年至 2016 年间的 47 例连续 ATL（急性，n=23；淋巴瘤，n=14；慢性，n=8；惰性，n=2）患者（中位年龄 51 岁，96%为非裔加勒比人）。急性、淋巴瘤和惰性（慢性和亚临床）形式的 3 年总生存率（OS）分别为 15.8%、11.3%和 85.7%。在侵袭性亚型中，20 名患者接受了高剂量齐多夫定和干扰素 α（AZT-IFNα）作为一线治疗，总缓解率（ORR）为 39%（完全缓解 [CR] 33%），17 名患者接受了化疗，ORR 为 59%（CR 50%）。38 名患者发生了 95 例感染，其中大多数为急性亚型（n=73/95；77%）。在随访期间，接受一线化疗或一线 AZT-IFNα 治疗的患者中，分别有 74%（n=14/19）和 89%（n=24/27）的患者发生感染。64 例（67%）的感染得到了微生物学证实。其中，侵袭性真菌感染（IFI，n=11）包括 2 例卡氏肺孢子虫肺炎、5 例侵袭性曲霉病和 4 例酵母血症。IFI 仅发生在急性亚型患者中，这些患者大多接受了 AZT-IFNα（n=10/11）治疗，并经历了延长（>10 天）的 4 级中性粒细胞减少症。发生 IFI 的侵袭性亚型患者的 OS 低于未发生 IFI 的患者（中位 OS 5.4 个月与 18.4 个月，p=0.0048）。即使在现代，ATL 患者的预后仍然较差。此外，感染率高会影响其管理，尤其是那些接受 AZT-IFNα 治疗的患者。
