Groiss Christina, Kreissl Stefanie, Strassl Irene, Saini Olga, Wipplinger Dagmar, Milanov Robert, Kaynak Emine, Hasengruber Petra, Aichinger Christoph, Nocker Stefanie, Bauer Thomas, Buxhofer-Ausch Veronika, Machherndl-Spandl Sigrid, Binder Michaela, Nikoloudis Alexander, Girschikofsky Michael, Petzer Andreas, Weltermann Ansgar, Clausen Johannes
Division of Hematology with Stem Cell Transplantation, Hemostaseology and Medical Oncology, Department of Internal Medicine I, Ordensklinikum Linz Elisabethinen, Fadingerstrasse 1, 4020 Linz, Austria.
Medical Faculty, Johannes Kepler University Linz, Altenberger Strasse 69, 4040 Linz, Austria.
J Clin Med. 2025 Apr 19;14(8):2816. doi: 10.3390/jcm14082816.
T-prolymphocytic leukemia (T-PLL) is a rare lymphoid neoplasm with particularly poor prognosis. Although it is no longer recognized as a distinct entity by the World Health Organization (WHO), B-prolymphocytic leukemia (B-PLL) comprises conditions with unfavorable outcomes. Both diseases most frequently affect patients in the seventh decade of their lives. Allogeneic hematopoietic stem cell transplantation (alloHSCT) significantly improves outcomes for selected PLL cases, as shown by several, mostly retrospective, analyses. In this article, we provide a review of existing PLL analyses, followed by a summary of cases treated at our center. We describe outcomes of six T-PLL and three B-PLL cases receiving alloHSCT at our institution between 2015 and 2022. Despite a post-transplant 4-year cumulative relapse incidence of 61% in our T-PLL series, the median OS was 78 months, because relapse therapy was remarkably successful. All B-PLL patients are alive and relapse-free, with a median follow-up of 54 (range of 11-74) months. A poor pre-transplant Karnofsky performance status (KPS) (≤ 80%) and an HCT comorbidity index (HCT-CI) of ≥3 were significantly associated with post-transplant mortality. The comparatively favorable outcomes in our case series underline the increasing value of alloHSCT in PLL in the current era, as it offers a prospect of cure in selected patients with otherwise very poor prognosis.
T 原淋巴细胞白血病（T-PLL）是一种预后特别差的罕见淋巴样肿瘤。虽然世界卫生组织（WHO）不再将其视为一个独特的实体，但 B 原淋巴细胞白血病（B-PLL）包含预后不良的情况。这两种疾病最常影响七十岁左右的患者。多项分析（大多为回顾性分析）表明，异基因造血干细胞移植（alloHSCT）可显著改善部分 PLL 病例的预后。在本文中，我们对现有的 PLL 分析进行了综述，随后总结了我们中心治疗的病例。我们描述了 2015 年至 2022 年期间在我们机构接受 alloHSCT 的 6 例 T-PLL 和 3 例 B-PLL 病例的预后情况。尽管我们的 T-PLL 系列中移植后 4 年累积复发率为 61%，但中位总生存期为 78 个月，因为复发治疗非常成功。所有 B-PLL患者均存活且无复发，中位随访时间为 54（11 - 74）个月。移植前卡诺夫斯基表现状态（KPS）差（≤80%）和造血细胞移植合并症指数（HCT-CI）≥3 与移植后死亡率显著相关。我们病例系列中相对较好的预后突显了在当前时代 alloHSCT 在 PLL 中的价值日益增加，因为它为部分预后原本非常差的患者提供了治愈的希望。
Zotero 插件