Guillaume Thierry, Beguin Yves, Tabrizi Reza, Nguyen Stéphanie, Blaise Didier, Deconinck Eric, Redjoul Rabah, Cornillon Jérôme, Guillerm Gaëlle, Contentin Nathalie, Sirvent Anne, Turlure Pascal, Salmon Alexandra, Huynh Anne, François Sylvie, Peffault de Latour Régis, Yakoub-Agha Ibrahim, Mohty Mohamad
CHU Hôtel Dieu, Nantes, France.
Eur J Haematol. 2015 Mar;94(3):265-9. doi: 10.1111/ejh.12430. Epub 2014 Sep 13.
T-prolymphocytic leukemia (T-PLL), a rare aggressive mature T-cell disorder, remains frequently resistant to conventional chemotherapy. Studies have suggested that allogeneic hematopoietic stem cell transplantation (HSCT) might possibly serve to consolidate the response to initial chemotherapy. The current report summarizes the outcome of 27 T-PLL cases identified in the registry in French Society for stem cell transplantation (SFGM-TC). Prior to HSCT, 14 patients were in complete remission (CR), 10 in partial response, three refractory, or in progression. Following HSCT, 21 patients achieved CR as best response. With a median follow-up for surviving patients of 33 (range, 6-103) months, 10 patients are still alive in continuous CR. Overall survival and progression-free survival estimates at 3 yr were 36% (95% CI: 17-54%) and 26% (95% CI: 14-45%), respectively. The relapse incidence after HSCT was 47% occurring at a median of 11.7 (range, 2-24) months. Overall cumulative incidence of transplant-related mortality was 31% at 3 yr. These results suggest that HSCT may allow long-term survival in patients with T-PLL following induction treatment; however, it is associated with a significant rate of toxicity.
T 原淋巴细胞白血病(T-PLL)是一种罕见的侵袭性成熟 T 细胞疾病,对传统化疗常常耐药。研究表明,异基因造血干细胞移植(HSCT)可能有助于巩固对初始化疗的反应。本报告总结了法国干细胞移植协会(SFGM-TC)登记处确定的 27 例 T-PLL 病例的结果。HSCT 前,14 例患者处于完全缓解(CR),10 例部分缓解,3 例难治或疾病进展。HSCT 后,21 例患者达到最佳反应 CR。存活患者的中位随访时间为 33(范围 6 - 103)个月,10 例患者仍存活且处于持续 CR 状态。3 年时的总生存和无进展生存估计分别为 36%(95%CI:17 - 54%)和 26%(95%CI:14 - 45%)。HSCT 后的复发率为 47%,中位复发时间为 11.7(范围 2 - 24)个月。3 年时移植相关死亡率的总体累积发生率为 31%。这些结果表明,HSCT 可能使诱导治疗后的 T-PLL 患者获得长期生存;然而,它与显著的毒性发生率相关。